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					CM-MSK Exam 2 Notes
Shoulder Pain
   1. Pain Patterns
          a. Anterior Pain: biceps tendinitis, AC joint
          b. Lateral deltoid pain: Referred from bursa
          c. Pain with clicking: labral, bursa
          d. Superior pain: AC joint/scapula/cerv spine
          e. Radicular symptoms (nerve root problems): lightning/parasthesia, cerv spine or shoulder
             dislocation
   2. Differential Diagnosis for Shoulder Pain




   3. Case 1: 53yo teacher comes in with 3 weeks of R shoulder pain. Notes that it started the day after
      painting her bedroom ceiling. Pain is achy and radiates to lateral arm. Flared up by overhead
      activity and worse when she lays on it at night. Denies trauma. Pain severe to interrupt tennis
      match. Difficulty writing on blackboard and tennis serve. (Important: pain that wakes you up from
      any position more likely to be rotator cuff)
   4. Key history for “Bursitis” aka Subacromial impingement (btw acromion and greater tuberosity)
          a. Pain in shoulder, top, posterior or radiating to lateral deltoid area
          b. Worse after overhead activity
          c. Onset after repetitive activity
          d. Can’t fasten bra
          e. Pain wakes up if roll over on it
   5. Important Physical Findings
          a. No atrophy
          b. Limited ROM (+ arc sign 70-120degrees) – how is scapula moving?
          c. All RC muscles weak –due to pain or RC problem?
          d. Join is stable
          e. + Hawkins sign: examiner exerts internal rotation of humerus with 90degrees of forward
              flexion of 90degrees of elbow flexion; positive test is reproduction of pain (pinches bursa)
   6. Management
          a. Assessment
          b. Plan
                   i. Xray: check for fx, arthritis
                  ii. Injection: doesn’t change course of disease, but can FACILITATE rehabilitation
                 iii. Anti-inflammatory drugs
                 iv. PT: to “re-learn” the correct muscle-firing patterns
                  v. Posture: Slump=scapula rotate outside, mess up muscles
7. From impingement to RC tear
        a. Normal  Grade 1 (months)  Frank RC tear (swollen bursa wears hole in rotator cuff)
8. Case 2: 68yo presents with several months of R shoulder pain, post. And deep. Hurts all the time,
    esp after activity. When he lays down at night, throbs and wakes him from sleep. No trauma and
    otherwise healthy. He has pain on attempting to initiate abduction of the shoulder (3/5). How is
    this different than Case 1?
        a. Initiation of motion more of a problem, longer, age
        b. Key Historical features
                 i. Atrophy may be present: supraspinatus (with difficulty initiating movement)
                ii. ROM: passive normal, active abnormal
               iii. Strength: specific RC weakness (subscapularis strong, but supraspinatus weak and
                    possibly infraspinatus as well)
               iv. Stability
                v. Hawkin’s Sign (may be causing night pain)
        c. Physical exam:
                 i. Atrophy: because of muscle OR nerve supply
                        1. Suggest RC injury
                ii. Relate historical findings to physical findings mechanically
        d. Management
                 i. Diagnosis: RC Tendinitis
                        1. How to distinguish from impingement? Injection fixes pain/movement for
                           bursitis; but for RC injury, use injection to see if pain goes away BUT MUSCLE
                           WEAKNESS WILL STILL REMAIN
                ii. Plan
                        1. PT: Retrain RC muscles that remain (so they pick up the slack)
                        2. Anti-Inflamm: Caution! GI bleeds
                        3. Imaging: MR arthrogram (age dependent)
                        4. Referral: If RC cannot be used, or if pain cannot be eliminated
9. Case 3: 13yo with pain at supero-lateral aspect of shoulder. Started after a hard throw from the
    outfield a few days ago. Achy, worse at night, can’t through, weak 6-8/10. Arm just “feels dead.”
        a. Little Leaguer’s Shoulder: Overuse due to repetitive throwing. Widening of proximal
            humeral growth plate (RC muscles attach on greater tuberosity, traction with slowing).
            Adolescents 11-14 with open epiphyseal joint. Pain in lateral shoulder, deep aching and
            throbbing with throwing.
                 i. Etiology: time of rapid bone growth. Learning and over-practicing new pitches
                    (technical errors, pitch counts)
        b. Physical Exam
                 i. Pain on palpation (lateral proximal humeral head)
                ii. Mild pain with external rotation
               iii. Pain with infraspinatus and supraspinatus testing
               iv. Confirm with radiographs (tuning fork)
        c. Management for growth plate fx of proximal humerus
                 i. Plan:
                        1. Sling
                        2. PT
                        3. Pain control (careful, don’t want to stunt growth)
10. Case 4: 48yo diabetic complains of progressive pain and limited ROM with lifting arm over
    head for past 3 months. She is now really having trouble even brushing her hair. Pain over her
    whole shoulder, achy 4/10, associated with limited ROM.
11. Adhesive Capsulitis
        a. Progressive loss of ROM
           b. Three stages: Painful (1-2 yrs), Adhesive (4-6 mos), Recovery stage
           c. Adhesions in joint
           d. Associated with medical conditions (diabetes, scleroderma, thyroid, CVA, mastectomy,
               RA, lung cancer, TB, COPD, MI)
           e. 7-15% permanently lose motion; little disability
           f. Profile and Symptoms
                    i. Patients >40
                   ii. 15% of patients bilateral
                  iii. May follow trauma
                  iv. Complaints: vague pain in shoulder, inability to retrieve wallet or undo bra straps
                   v. Symptoms: progressive stiffness, pain if rolling over on joint, overuse of RC tendons
           g. Tx:
                    i. PT
                   ii. Intra-articular injection (lidocaine and steroids, capsule restriction)
                  iii. Manipulation under anesthesia followed by PT
                  iv. Freeze/thaw cycle takes about 2-3 years
   12. Case 5: 68yo with several months of R progressive loss of motion. Stiff in the morning for about
       an hour, painful at extremes of motion. Pain is deep and achy. Hurts all the time; loosens after
       activity. How is it different from 1 and 2?
           a. Generalized OA (Xray findings, other sites e.g. hands)
                    i. Shoulder pops with limited ROM
                   ii. RC strong but a little muscle wasting noted
                  iii. Joint stable- in fact, very little glide noted
                  iv. No abnormal blood tests
           b. Tx: injection, acetaminophen, PT, joint replacement eventually
   13. Bonus!!! 59yo getting his golf clubs and feels pop, arm looks like Popeye.
           a. Biceps tendon rupture: Anterior pain but usually a painless “pop.” Mostly long head
                    i. Exam: pain at bicipital groove, weakness of supination, arm flexion strength less
                       affected
                   ii. Complication is supination weakness
Traumatic Shoulder Injuries
    1. How to Injury Your Shoulder
           a. Falling on it/Axial Load: humeral fracture, clavicular fracture, AC separation
           b. Force applied to abducted shoulder: capsular injury
           c. FOOSH: dislocation, depending on how you fall
    2. Static Stabilizers
           a. Glenoid (convex, pear shaped, humeral head at least 3x, <1/3 humeral head in contact at
               any time.
           b. Glenoid labrum: cartilage rim around edge. Makes shoulder socket a little deeper, more
               flexible.
                    i. Superior portion: relatively mobile
                   ii. Inferior: immobile
           c. Capsule/Ligaments: “shrink wrap” that holds humeral head in place like wheel/axel.
                    i. Ligaments:
                           1. Superior glenohumeral
                           2. Middle glenohumeral
                           3. INFERIOR GLENOHUMERAL (most important ligamentous stabilizer when
                              shoulder is abducted and externally rotated)
                 ii.
         d. Joint Cohesion: limited volume of fluid, airtight
         e. Intraarticular pressure: negative pressure holds joint together, hole in capsule leads to
             instability
3.   Dynamic Stabilizers
         a. Anterior: Subscapularis, long head of biceps tendon (not RC muscle)
         b. Posterior: supraspinatus, infraspinatus, teres minor
4.   Scapulothoracic and Glenohumeral Motion
         a. Scapula positions glenoid for stability in all positions (upward rotation to allow overhead
             activity)
                  i. First 30-45degrees of Abduction has little movement of scapula, after that RATIO IS
                     2:1
5.   Scapulothoracic Stabilizer
         a. Scapular Retractors: rhomboids, trapezius
         b. Scapular Protractors: serratus anterior, pectoralis minor
         c. Scapular Rotators: trapezius (upper and lower fibers), serratus anterior (lower fibers)
         d. Relate to previous OMM labs with thoracic diagnosis
6.   Case 1: 44yo complains of deep click and pain with overhead shoulder activity pain. Pain
     getting worse, occasional night pain that wakes from sleep. Pain vague and 3/10, radiates to
     lateral deltoid. Plays racquetball. May have started after a fall.
         a. Exam: no atrophy, full ROM with clicks noted superiorly, strength 4/5 RC, slight increased
             laxity, shoulder not unstable.
                  i. + Obriens (for labral pathology)
                 ii. +Hawkins
         b. Tx:
                  i. Injection: for diagnostic purposes, get bursa out of the picture (see if exam changes)
                         1. If popping is bursa and you numb it, it doesn’t hurt anymore! But this case
                             still has pain because it is a labral tear
                 ii. PT: retrain, posture
                iii. Anti-inflamm
                iv. MR arthrogram if non-surgical management unsuccessful (labral tear – fluid leaks
                     out of tear site)
                 v. Referral to orthopedic surgeon (arthroscopic repair of labral tear)
       c. “Best” Instability Tests –Anterior Release best sensitivity and specificity




7. “SLAP” lesions: Superior Labral tear in Ant-Post plane
        a. Most common cause of instability-type injury such as dislocation
        b. Multiple types: concept most important that labrum is separated from underlying glenoid,
            may involve biceps tendon origin
8. Bicipital Tendonitis: “RED FLAG diagnosis”
        a. True bicipital tendonitis extremely rare
        b. Usually due to impingement or a labral tear/SLAP lesion
        c. Find and treat the underlying cause
9. Case 2: “Shoulder Dislocation.” Baseball player dives for a ball and lands on his throwing arm.
    Immediate pain and is unable to move his right shoulder. Bulge is noted in anterior aspect of his
    jersey.
        a. Check sensation, pulses.
        b. Shoulder Dislocation
                i. Anatomical considerations
               ii. Etiology and types
              iii. Evaluation
              iv. Relocation techniques
               v. Complications/assoc. pathology
              vi. Tx
10. Shoulder Instability (differentiate instability based on mnemonics)
        a. TUBS
                i. Traumatic
               ii. Unidirectional dislocation with an association
              iii. Bankart lesion, likely to respond to
              iv. Surgery
        b. AMBRI
                i. Atraumatic
               ii. Multidirectional
              iii. Bilateral instability, likely to respond to
              iv. Rehab but may require
               v. Inferior capsular shift
11. Type of dislocation
        a. Anterior: subcoracoid. Abduction and external rotation, direct blow to posterior shoulder,
            FOOSH
       b. Posterior: Arm forward flexed, internally rotated, adducted with posterior directed force;
          secondary electric shock/seizure; direct (car) or repetitive trauma (football) to anterior
          shoulder
12. Physical Exam
       a. Anterior Dislocation
                i. Hollow under acromion posteriorly
               ii. Humeral head palpable anteriorly
              iii. Arm in abduction and external rotation
              iv. Decreased internal rotation
       b. Posterior
                i. Prominent coracoid process
               ii. Flattened anterior aspect of shoulder
              iii. Prominence of posterior shoulder
              iv. Arm adducted and internall rotated
               v. Cannot externally rotate
       c. Inferior
                i. Arm elevated and hyperabducted (resting on back of head)
13. General Laxity




        a.
14. Instability Testing
        a. Anterior Apprehension Test (variation of “relocation test”)




                                                                         Relocation
15. Axillary nerve evaluation                                            Test
       a. Intact sensation over lateral deltoid, full arm extension strength (isolates posterior deltoid
           muscle)
16. Radiographs
       a. Hill-Sachs lesion: posterolateral humeral head fractures as it impacts the anterior inferior
          glenoid rim (A)




       b. Bankart Fracture: anteriorinferior glenoid rim fractures as humeral head dislocates.
          Labrum has pulled a piece of bone away as part of tear




17. Complications: fractures, muscle injury, vascular injury, nerve injury (axillary), recurrent
    instability
18. Tx: Goal to restore normal function allowing return to athletics. Younger than 25 usually surgery,
    older usually rehab
        a. Pain control
        b. ID associated injuries by clinical exam and Xray
        c. Brace: immobilization in external rotation 2-3 weeks, places posterior-superior labrum in
           anatomic approximation
        d. *** Younger you are, more likely you are to have recurrent dislocation. GET SURGERY***
19. Case 3: Separated Shoulder. 140-pound wrestler thrown to the mat with his arm in adduction.
    Lands on superior lateral aspect of acromion and feels pop. On exam, he is in a lot of pain. You
    detect swelling and pain over AC joint. It is worse with crossed arm adduction and resisted
    abduction.
        a. Exam: look for AC/SC joint deformity, Palpate SC, length of clavicle AC, acromion, scapular
           spine, ROM – quality of scapula-thoracic glide and total motion, cross-over and “Chuck
           Norris” test to load AC joints
20. AC joint: acts as strut to support scapula; plane, synovial, small cartilage plate (meniscus) btw
    acromion and clavicle
          a. Superior AC ligament: controls horizontal stability and is most important ligament in
             stabilizing AC joint for normal daily activities. Conoid and trapezoid ligaments control
             anterior and posterior rotation of clavicle in overhead activities
   21. Separations




          a. Manage: Ice, NSAIDs, rest, sling for comfort, early ROM, good return of function
   22. Clavicle fractures
          a. Dx by history of MOI (fall on shoulder, FOOSH)
          b. Inspection to see loss of normal clavicular contour
          c. Palpation: crepitus
          d. Extreme pain with ROM over 90degrees or in adduction
          e. Xray
          f. Middle 1/3 fractures
          g. New emphasis on restoring anatomic position and length


Non-Surgical Injuries of Hand and Wrist
   1. Exam
          a. Inspection, Palpation, ROM, Strength Testing, Special tests
   2. Trauma vs. Overuse
   3. Carpal Tunnel: OVERuse, Compression of MEDIAN nerve, Pain/numbness
          a. Test: Tinel’s (tap on median nerve) and Phalen’s (pain, numbness and/or tingling over
             median nerve dist.
          b. Chronic pain, muscle atrophy in rare cases, nerve conduction studies
          c. Tx: OMT, NSAIDs, night splints, OT, injection, surgery
   4. DeQuervain’s Tenosynovitis: OVERuse, Pain at distal radius, involves tendons of EPB and APL
      (snuffbox)
          a. Chronic pain, usually no nerve involvement, point tender to touch over wrist
          b. Test: Finkelstien’s Test (fishing pole)
          c. Tx: OMT, rest, bracing, NSAIDs, injection, iontophoresis, OT
   5. Intersection Syndrome: OVERuse, inflammation of ECRB and EPL
          a. Involves more prox and dorsal wrist and not snuffbox, palpation and knowing anatomy,
             “Squeakers wrist”
          b. Tx: rest, NSAID, brace, injection
   6. Trigger Finger: ganglion cyst forms on flexor tendon and gets caught under A1 (snap or get stuck)
          a. Pain in mechanical symptoms
          b. Tx: Injections, Surgery
   7. Guyon’s Canal Syndrome
          a. Irritation of ulnar nerve at Guyon’s canal, “Biker’s Wrist,” “Handlebar palsy,” cyclists
          b. Numbness and tingling along ulnar nerve distribution (pink, 4th finger)
          c. Tx: modify hand position or padding, imaging with trauma, OT, injection
   8. Mallet Finger: traumatic injury to finger after axial load, DISTAL EXTENSOR TENDON is ruptured,
       pt cannot extend finger
           a. Get XRAY so you know it’s not an avulsion fx
           b. Tx: Grade 3 involves more than 50% fx may be more aggressive, but STACK SPLINT 24/7
               for minimum 6 wks
   9. Jersey Finger: FDP tendon rupture at distal phalanx (volar aspect)
   10. Skier’s Thumb: disruption of ulnar collateral ligament from valgus force (falling/trauma)
           a. Grade 1: sprain, 2: partial tear, 3: full tear
           b. Test: Flex MCP joint to 90 degrees and use valgus force
           c. Tx: 1&2 – nonoperative, 3 – reconstruction
   11. Sprains and Strains
           a. Very common
           b. Dx of exclusion – eval for specifics first
           c. RICE
           d. Follow-up as needed
           e. Trust your clinical and anatomical knowledge
           f. Don’t Xray every pt!
           g. Use PT/OT
           h. Don’t immobilize too long
   12. Dislocations
           a. Reduce when appropriate (fingers more appropriate to reduce in field than wrist)
           b. Pre- and post- reduction films helpful
           c. Fx
           d. F/U
   13. Radicular patterns from proximal sources
           a. Good rule: Examine joints above and below area of pain
           b. Ex: Ulnar nerve irritation at elbow, cervical spondylosis
   14. Tips:
           a. Listen to pt
           b. Don’t xray all the time
           c. If unsure, immobilize
           d. EDUCATION of pts, Explain things
Surgical Treatment of Forearm and Wrist Trauma
   1. Describe the indications for surgical treatment options of two-bone forearm fractures and distal
      radius fractures.
         a. Displaced, transverse, two-bone forearm shaft fractures
         b. Comminuted, angulated, displaced fx of the distal radius
         c. Indications
                  i. Open fractures
                 ii. Increased risk of loss of reduction or “slipped” reduction
                iii. Fx of both bones of the forearm
                iv. Comminution of distal radial fracture fragments
                 v. Intra-articular fx
                vi. Multiple fx
   2. Ex-Fix
         a. Indications: Open fx, severe soft-tissue
         b. Contra-indications: unreliable patients
         c. Advantages: rapid, cheap, modest risk of infection, outpatient management, removal of
             implant w/o need of anesthesia
      d. Disadvantage: Pin-track infection, nerve damage, less comfortable, high rate of non-union,
          not fully qualified as definitive tx, risk of damage to radial nerve (ramus superficialis)
3. C/R internal fixation
      a. Indications: soft tissue does not allow casting/bracing or ORIF of both, cosmetical reasons
      b. Contra: critical local soft-tissue conditions at intended insertion site
      c. Adv: rapid surgical procedure, less vascular compromise
      d. Dis: need of image intensifier, no exact control over rotation (BEST FOR ELDERLY, NOT
          YOUNG)




      e.
4. ORIF
      a. Indications: all closed forearm fractures, 2o procedure after failure of conservative tx and
         change of procedure following CREF, delayed union or nonunion, open fx (Gustillo 1 and 2),
         chain injury, definitive management of poly-trauma
      b. Contra: critical soft-tissue condition
      c. Adv: pt’s comfort, anatomical reduction and early functional tx
      d. Dis: surgical risk
      e. Surgery for radius: dissect interval btw brachioradialis and FCR. Radial artery should be
         under brachiorad and between two tendons in distal part. Retract superficial radial nerve
      f. After: splint, therapy
      g. Remove implant: removal is controversial
              i. Removal only in symptomatic pts, possibly only on ulna
             ii. Removal no earlier than 2 yrs after osteosynthesis
            iii. Minimally invasive removal by stab incisions for screws and plate
5. C/R with percutaneous pins (SIMPLE fx)
      a. Adv: quick and cheap, easy hardware removal, less finger stiffness
      b. Dis: pin irritation, infection, only on relatively simple fx
      c. Aftercare: cast for 6wks then remove the pins
      d.
6. C/R with traction cast (COMMINUTED fx)
      a. Adv: quick and cheap, useful on comminuted fx
      b. Dis: hardware complications, nerve damage d/t pins
      c. Aftercare: cast for 8wks, remove pins, PT
7. C/R with Distraction Plate Fixation
      a. Adv: fixation is concealed, solid fixation possible through ligamentotaxis and diaphyseal
         bone
      b. Dis: hardware complications, adhesions and finger stiffness, second procedure to remove
         hardware
      c. Aftercare: begin PT immediately, remove plate when fx has healed




      d.
8. C/R with external fixation
      a. Indications: open fx, closed fx with compromised soft tissues
      b. Contra: severely comminuted, osteoporosis, significant metaphyseal defect after restoring
         radial length
      c. Adv: reduced risk of infection at fx site, lower risk in significant soft-tissue injury
      d. Dis: require supplementary percutaneous pinning, pin-track infection, osteo-necrosis at pin
         sites, radial nerve injury, risk of re-displacement, unable to accurately control dorsal-ulnar
         fragments, risk of tendon trans-fixation
9. ORIF with plate and screws
      a. Indications: small articular fragments, impacted fragments, persistent displacement
         following other methods, instability, re-displacement, active patients
      b. Contra: significant closed skin injuries
      c. Adv: anatomical reduction, stability, early motion, ID of assoc inter carpal ligament injury
      d. Dis: nerve injury, tendon irritation, possible need for later implant removal
           e.
Injuries to Elbow and Forearm
   1. History
         a. OLDCARTS
         b. Age
         c. Occupation
         d. Hand dominance
         e. Specific MOI
         f. Functional loss
         g. Past medical/ family history
         h. REMEMBER: referred pain, neuro symptoms, examine joint above and below
   2. Overuse:
         a. Bicep tendonitis, triceps tendinits, olecranon bursitis, nursemaids, MCL sprain, medial
             apophysitis
   3. Lateral Epicondylitis: aka Tennis elbow
         a. Faulty mechanics, poorly fitted equipment, repetitive job at work
         b. Difficulty with wrist extension
         c. Xray
         d. Tx: rest and ice, forearm splint, OMM, rehab, PRP/autologous blood, steroid injection,
             prolotherapy, sugery LAST resort
   4. Medial epi: aka Golfer’s Elbow
         a. Faulty mechanics, poorly fitted equipment, repetitive job at work
         b. Increased pain with wrist flexion and forearm pronation
         c. Negative Tinel’s test at cubital tunnel
         d. Xray
         e. Tx: rest and ice, splint, OMM, rehab, PRP/Autologous, steroid injection, prolotherapy,
             surgery LAST resort
   5. MCL Sprain
         a. Most important stabilizer of valgus stresses (20-130 degrees)
      b. Repetitive valgus stress causes microtears or complete ruptures
      c. Gradual onset of medial pain (tenderness over the humeroulnar joint, sublime tubercle),
         relieved by rest, may have signs of ulnar nerve irritation
      d. Pain increases by manual valgus stress (Valgus stress test, Moving valgus stress test,
         milking maneuver)
      e. Tx: rest, NSAIDs, PT, strengthening/stretching, PRP/autologous blood, prolo, surgery in
         competitive throwing or persistent laborers
6. Medial Apophysitis
      a. Growth plates still open, partial or complete tear off the medial epicondyle
      b. Pain at medial epicondyle with possible swelling/bruising
      c. Xray (typically want to get b/l, widening of apophyseal line)
      d. PREVENTION
      e. Tx: rest, NSAID, rehab
7. Dermatomes




8. Median Nerve Injury: typically by blunt trauma, penetrating wounds
       a. Innervates the thenar compartment, allowing for fine control of the pincer grip (APE
           HAND)
       b. Motor and sensory loss to thumb, index finger, and middle finger
       c. Prevents full flexion as the ring and little fingers flex normally
9. Anterior Interosseous Syndrome
       a. Branch of median nerve, mostly motor
       b. Strenuous or repetitive elbow motion exercises, fx, lacerations, blood draws, trauma
       c. Nerve often compressed by tendonour origin of deep head of pronator teres
       d. Presents 1-2days if injured
       e. Weakness or loss of flexion of DIP joint of thumb index finger
       f. EMG/NCV (technically difficult, false+ is <3 weeks of onset
       g. Tx: lifestyle modification, splinting, NSAIDs, PT, OMT, surgical decompression is over 6
           months
10. Flexor-Pronator Mass Syndrome: purely sensory
       a. Median nerve becomes trapped between heads of pronator teres muscle
       b. Symptoms: pain in volar forearm, paresthesia thumb, index, middle and part of ring finger
       c. Mech: repetitive pronation, anomalous anatomy
       d. Resisted flexion of FDS tendon of index/middle finger (PALPAL SIGN)
       e. Resisted pronation of forearm reproduce symptoms
       f. Negative Tinel’s Phalen’s test at wrist
       g. Conservative tx
11. Radial nerve injury: “Saturday night palsy”
       a. Induced by stab wounds to chest; humerus fx, lead poisoning
       b. Radial nerve divides into the superficial and the deep branch (posterior interosseous
           nerve) at the lateral epicondyle
       c. Superficial branch is purely sensory
       d. Injury proximal to the branching leads to wrist drop/sensory loss
12. Ulnar Nerve injury
       a. Innervates many intrinsic muscles for hand, extrinsic for flexion of ring and little fingers to
           allow for grip
       b. Sensory loss noted in palmar and distal dorsal surfaces of the little finder and medial half of
           ring finger
       c. Muscle wasting/atrophy of 1st dorsal interosseious
       d. “CLAW HAND”
13. Radial head subluxation/dislocation “NURSEMAID’S ELBOW”
       a. Sudden traction on extended and pronated arm
       b. Radial head slips under annular ligament, distal attachment weaker
       c. Most common <6 yrs old
       d. Child doesn’t use arm, hold in flexed position against body
       e. No swelling or deformity, no surgery
14. Forearm Fractures
       a. Falls
       b. PRICE: protection, RICE
       c. Goals:
                i. Define MOI
               ii. Delineate extent of fx
              iii. ID any other injury
       d. Neuro exam
                i. Assessment of capillary refill
               ii. Pulses in radial and ulnar arteries
       e. Sensory and motor function of hand and wrist
15. Posterior Fat Pad sign: for occult fractures
16. Supracondylar fx: most common children’s elbow fx
       a. Extension type injury, some neuro injury, HIGH MALUNION
17. Dislocation of Elbow (w/o major fx)
       a. Splint then hinged brace
       b. Sometimes occurs with avulsion fx of medial epicondyle
       c. Surgery seldom required unless medial epicondyle is stuck in joint
18. Olecranon Fx
       a. Usually from direct trauma in flexed elbow (intra-art, managed surgically)
       b. Avulsion of triceps tendon (extra-articular, managed conservatively)
19. Coronoid Fx
       a. RARE
       b. Acts as a buttress
       c. 10-15% of elbow dislocations
       d. <5mm displacement and a stable elbow can be treated conservatively
20. Radial head fx
       a. FOOSH
       b. Forearm movements painful and limited
       c. Tenderness elicited over radial head
       d. Tx generally conservative with sling and early mobilization
21. Midshaft Fx
       a. 2-bone, Night-stick, Monteggia fx
   22. Distal Radial Fx
          a. Colles Fx
          b. “Dinner Fork deformity”
          c. Common in younger adults or older persons
          d. FOOSH
          e. Closed manipulation and casting




          f.
          g. Chauffer’s




   23. Distal Ulnar Fx
          a. Styloid fx
          b. Assess distal RUJ stability
          c. Intimately related to TFCCj
Surgical Hand Shtuff
   1. Flexor Tendon Injuries
         a. Treating injuries in lumbrical region heal well, but in the “No Man’s Land” (between A1 and
             A3 or 4) have very little room for anything, hard healing, most distal (Zone 1), tendon can
             be re-attached easily
         b. Flexor digitorum profundus goes through Chiasma of camper (as superficialis splits)
         c. NOT ALL ARE THE SAME, think of where they occur!
   2. Diagnosis
         a. H&P – MOI – flexion =injury distal to skin laceration; MOI – extension, close to skin
             laceration; MOI – natural resting position, composite flexion increases radial to ulnar
                  i. FDP only – MCP and PIP joints okay, DIP
                 ii. FDP and FDS – flat in extended position
                iii. Test each joint separately
                iv. Passively manipulate wrist through F/E tenodesis
        b. Order Xray to R/O boney pathology
        c. IV antibiotics and tetanus
        d. To OR, Bruner’s (zig-zag incisions)
3.   Flexion of fingers demonstrates loss of active movement at the tip of the ring finger despite
     normal joint mobility due to a closed FDP rupture
        a. Primary repair within 12hrs or so, Delayed repair for whatever reason, staged
            reconstruction after way too long w/o repair
        b. Complications: rupture, adhesions
        c. PT: modified Kleinert protocol, Duran, Indianapolis protocol, Delayed Mobilization in Zone
            I-V injuries
4.   Extensor tendon injuries
        a. Indications: tendon laceration greater than 50%, etc.
        b. Contra: Skilled physician unavailable, contaminated injury, bone fracture, open joint space,
            overlying skin loss
        c. Compared to flexor, they are less robust
5.   Boutenniere Deformity – extensor tendon
        a. Where PIP pushes through extensor mechanism. Central slip of extensor tendon inserts at
            base of middle phalanx. This can be disrupted (laceration, etc.). When the central slip pops,
            lateral bands still attach. As soon as lateral band goes volar, pull causes distal phalanx to
            extend and middle phalanx to flex (zig-zag deformity)
                 i. SPLINT to maintain PIP extended, allow DIP to flex. If it doesn’t work, surgery
        b. Hyperextension DIP, flexion PIP
6.   Swan – extensor
        a. Flexion DIP, hyperextension PIP
                 i. Mobilize lateral bands
7.   Mallet Fx: DP is no longer attached by extensor ligament
        a. Reduction: incision, intro of K-wire through DP medullary canal, reduce and drive K-wire
            into MP. Suture, tendon/bone to button.
        b. Zone I Extensor Tendon Injury; With or without boney injury
8.   Fingernail Problems
        a. Subungal hematoma (blood under fingernail)
        b. Split nail
        c. Pincer deformity
        d. Hooked
        e. Parts of Nail:
                 i. Eponychium – cuticle
                ii. Paronychium – border tissue around nail
               iii. Hyponychium – the “quick,” under the front of the nail
               iv. Growth rate: .1mm/day
        f. Paronychia: “runaround” infection, S. aureus
                 i. Eponychial
                ii. Hangnail/poor hygiene
               iii. Unilateral – incise, knife away from nail
        g. Felon: abscess of subcutaneous pulp
                 i. Penetrating FB, “sticks”
                ii. S. aureus
               iii. Antibiotics and drainage (where point of pus is)
               iv. Within confines of septum
9.   Carpal Tunnel Syndrome
          a. Exam points: Phalen’s test, reverse, Tinel’s, compression, Allen’s (2 vessels that supply
              hand; hand on both areas then release to see color change in skin), sensory, muscle
              atrophy, motor strength
Adult Hip Pain
   1. Hip is a region
          a. History: acute, chronic, overuse, traumatic; associated signs: pops or clicks, OLDCARTS
          b. Consider referred pain: SI dysfunction, Lumbar radiculopathy
   2. Hip Exam
          a. Inspect anterior/posteriorly (Watch Gait)
          b. Note asymmetry in iliac crest height, size of buttocks, or number/level of gluteal folds
          c. Palpate hips and pelvis with patient supine; note instability, tenderness, or crepitus.
          d. Examine ROM: active flexion, extension, hip flexion, ab/adduction, internal/external
              rotation
          e. Test muscle strength: knee in flexion and extension, ab/adduction
   3. Thomas Test
          a. Ilipsoas tightness (thigh off table)
          b. Rectus femoris tightness (knee flexion >90)
          c. Tensor fascia latae (knee lateral to ASIS)
          d. Iliotibial band (Foot ER)
          e. Key is to hold opposite knee tightly to chest
   4. FABERE/Patrick’s
          a. Flexion, Abduction, External Rotation
          b. Pain before SI joint is engaged (early ROM) indicates pain in acetabulum/femoral joint
          c. Pain after SI joint (late ROM) indicates SI as source of pain
   5. Ely’s Test
          a. Rectus femoris tightness
          b. Flexion of knee flexes or pulls hip off table
   6. Hibb’s Test
          a. Pt prone, flex knee to 90
          b. IR and ER hip while monitoring pelvis
          c. Can use monitoring hand to confirm engagement of SI joint
          d. Pain early=probably from hip; later=SI joint
   7. Scrub Test
          a. Pt supine and hip flexed
          b. Compress femoral head into acetabulum and maintain compressive force as you move hip
              through circular ROM. Reproduction of pain indicates intra-articular source of pain
   8. Leg Length Discrepancy
          a. Functional vs. anatomic
          b. Check below both malleoli after balancing/straightening pelvis
   9. Standing flexion test
          a. positive on the side where the thumb moves further superiorly. In a standing position the
              sacrum is relatively flexed and with flexion of the spine the sacrum moves without also
              moving the innominate bones. If SI restriction exists the sacrum will move the innominate –
              PSIS travels upward
          b. Seated flexion test is more specific for SI because lower extremity dysfunction is effectively
              removed
   10. Referred pain
        a.
11. Hip Pain Overview
        a. Sources: Hip joint, soft tissue, pelvic bones, SI joint, referred from lumbar
        b. Hip is one part of pelvic girdle (ilium, pubic ramus, sacrum) and contains 2 joints (SI and
           hip)
        c. Watch them walk!
12. Case: 83yo mother of nurse. Pain of gradual onset which started at anterior portion of hip but now
    some pain laterally. Originally relieved by Tylenol and rest, but now pain at night. Particular type
    of antalgic gait.
        a. Loss of articular cartilage at hip joint
        b. Etiology: trauma, infection, genetic, idiopathic
        c. Hx: gradual onset, anterior groin pain but may be butt or lateral thigh, see case paragraph.
        d. PE: limited ROM (internal rotation), then loss of flexion and extension, antalgic gait and
           abductor lurch; positive Trendelenburg
        e. Imaging: Xray
        f. Tx: Strengthening, PT for ROM, surgery
        g. DDx:
                 i. Lumbar Disc disease (normal hip motion), herniated lumbar disc (diminished knee
                    reflex, sensory changes)
                ii. Femoral cutaneous nerve entrapment (sensory changes, burning, normal motion)
               iii. Hip dysplasia – developmental
               iv. Osteonecrosis of femoral head (xray)
                v. Trochanteric bursitis (local tenderness, normal motion)
               vi. Tumor of pelvis or spine
              vii. Trendelenburg Test
13. Case: Ron is a 54 year old military veteran with a history of hip injury in the marines. He has a hx
    of obstructive lung disease and is on oral steroids frequently. He continues to smoke and drinks
    heavily. He now complains of groin pain which he has had for 5 months and it is getting worse.
        a. Ant Hip Pain: Osteonecrosis. Loss of trabecular bone in femoral neck usually during 3rd/4th
           decade
        b. Etiology: Trauma (hip dislocation or femoral neck fx), alcohol abuse, steroid use, RA, SLE,
           sickle cell, radiation, Crohn’s Caisson’s
        c. Hx: gradual onset, groin pain but may be butt or lateral to hip, may be sudden if femoral
           neck collapses
        d. PE: pain with internal/external rotation of hip and abduction. If fem neck has collapsed,
           they have pain with limited ROM, antalgic gait
        e. Imaging: XRAY AP pelvis and frog view; sclerosis or fem neck collapse
        f. Tx: Strength, PT, surgery
        g. DDX:
                 i. Fracture of femoral nexk (xray)
                ii. Lumbar disc (back pain and reflex changes)
               iii. Muscle strain (normal xray)
               iv. OA, septic arthritis (fevers)
14. Case: 48 year old has 2 weeks of deep achy left anterior hip pain. The pain is worse after being
    seated for prolonged periods and after sleeping. It is better with movement, however, it is sharp
    and “catches” him like it will “give way” with internal rotation and thigh extension. It started 2
    weeks ago abruptly when, while intoxicated, he was thrown from a mechanical bull. He was
    thrown several yards and landed with his left leg straight and “it jammed my hip.”
        a. PE: Mild ecchymosis resolving in the distal medial thigh.
        b. Palpation: no discreet area
        c. ROM: Pain with limited internal rotation and extension and a deep “click” on Thomas test
            with extension.
        d. Strength: 5/5 Quads, no atrophy
        e. Common Problems: Labral Tear
                 i. Etiology:
                ii. Tear of the fibrocartilaginous labrum usually due to high impact trauma.
               iii. Tear usually anterior labrum
               iv. Mechanism: Running, Hyperextension at hip, trauma
                v. History: Deep sharp anterior hip pain, deep clicking or snapping, sense of instability
               vi. Physical Findings: Anterior hip pain with hip into extension, pain with anterior
                    stress
              vii. Imaging: MRI
             viii. Treatment: Rest, Surgical repair
15. Case: 22 year old former collegiate soccer player from Mississippi moves to graduate school here
    in the new river valley. She has 4 weeks of anterior hip and thigh pain. The onset was gradual,
    shortly after moving here and starting to train for a marathon with her boyfriend. When
    questioned about distance, she states she’s been increasing only about 1 mile a week, until a
    month ago when she added hills and increased from 2 to 6 miles four times per week. At first her
    pain was only after running, but now her pain is constant, even at night.
        a. Stress Fx:
                 i. Etiology: Chronic overuse
                ii. usually compression sided
               iii. in Runners, Dancers, Recruits
               iv. Diagnosis delayed 5-13 weeks  risk of complications:
                v. AVN, nonunion, coxa vara, chronic pain
               vi. History: Anterior groin pain (87%), Weight bearing activity
                        1. Insidious onset
                        2. Training history
                        3. Recent increase
                        4. Hills/ mileage for femoral neck stress fractures
                        5. Prior stress fractures
              vii. Females
                        1. Menstrual cycles
                        2. Weight changes
                        3. Eating disorders
             viii. PE: Pain at extreme ROM (70%), Antalgic gait (22%), Palpation ant thigh (70%)
               ix. Hop Test
                        1. Femoral Neck
                x. Fulcrum Test
                        1. Femoral shaft
        b. Femoral Neck Stress Fx
                 i. Imaging
                        1. X-rays (healing); usually (-) for diagnosis
                        2. Bone-scan (+) 72 hours after injury
               ii. MRI
                       1. Similar sensitivity
                       2. Better specificity
              iii. Treatment
                       1. Strict rest (?!)
                       2. Non-weightbearing
                       3. Fixation
                              a. Tension vs compression side
       c. Avulsion Fx
                i. Etiology:
                       1. ASIS (sartorius),
                       2. AIIS (rectus),
                       3. Ischial (hamstring)
               ii. Mechanism:
                       1. Chronic, Acute concentric/eccentric
              iii. History:
                       1. Males, adolescents, local pain, limit motion
              iv. Physical Findings: tenderness, limit ROM & weakness on MMT
               v. Imaging:
                       1. AP pelvis, Oblique (iliac crest), CT
              vi. Treatment: Ice, stretch, gradual return (>2cm ORIF)
       d. Hip Pointer
                i. Etiology: Direct trauma ASIS, iliac crest
               ii. Mechanism: Collision in sports
              iii. History: Anterior/lateral pain after direct blow, pain localized, pain with laughing
              iv. Physical Findings: Local swelling, pain, ecchymosis
               v. Imaging: xray (R/o fracture)
              vi. Treatment: Pain control, NSAIDS, injections
       e. Osteotis Pubis
                i. Etiology: Inflammation, irritation of pubic symphysis
               ii. Mechanism: Excessive motion (muscle weakness), subluxation,  hip ROM, muscle
                   imbalance
              iii. History: Severe groin pain, diff standing, cutting
              iv. Physical Findings: Tender pubic symphysis, weak adductors, pain with one-legged
                   standing
               v. Imaging: AP pelvis, Stress (one-legged 3mm movement)
              vi. Treatment: Ice, Rest , Pain control, injection, OMT
       f. Sports Hernia
                i. Etiology: Several: groin muscles, conjoint tendon, Superficial inguinal ring
               ii. Mechanism: Tearing of soft tissue, overuse, trauma
              iii. History: Insidious pain, medial thigh, soccer, hockey, pain radiates to testes
              iv. Physical Findings: Localized pain, weak adductors, weak abdominal muscles, failure
                   to progress
               v. Imaging: MRI, CT (with contrast), US, may be negative
              vi. Treatment: Surgery (early?)
16. Case: Mary is a 41 yo teacher who developed increasing hip pain that is described as dull and
    aching. She feels pain in the groin and lateral thigh. She has no hx of trauma but does have SLE
       a. Inflamm Conditions
                i. Local manifestations of systemic disorders
               ii. Symptoms – dull aching pain in groin, lateral thigh, or buttocks. Pain is often
                   episodic with morning stiffness, improvement with moderate activity, and stiffness
                   of hip joint motion
              iii. Exam – pain with internal rotation and restriction
              iv. Diagnostics – AP pelvis and frog leg views may show decreased bone mineralization
                   or joint effusion; CBC, CRP,ANA; aspirate joint effusion and send for C&S, cell count
                   with diff, crystal analysis
               v. DDx:
                       1. ankylosing spondylitis, CPDD, infection, inflammatory bowel disease, Reiter’s
                           syndrome,RA, stress fracture, SLE, gout
              vi. Treatment – tx underlying condtion, NSAIDS, ASA; immunosuppressive agents;
                   surgery – total hip arthroplasty
17. Dislocation: of femoral Head from Acetabulum
        a. Etiology: Genetic, instability of joint
        b. Most are posterior; injury causes the hip to be adducted, flexed, and internally rotated. An
           anterior dislocation would leave the hip abducted, flexed, and externally rotated
        c. Mechanism: Direct blow with hip abducted (i.e. impact while slamming on brakes), non-
           contact
        d. History: Pain, inability to move, numbness
        e. Physical Findings: Short leg, hip adducted*, severe pain, inability to move; Evaluate sciatic
           nerve function by asking patients to move toes and ankle and checking sensation on
           plantar and dorsal aspects of foot
18. Hamstring Strain
        a. Etiology: Muscle tearing
        b. Mechanism: Acute overstretching, running, sprinting
        c. Factors: inflexible, fatigue, imbalance, incomplete rehab
        d. History: Local pain, deformity, popping sensation
        e. Physical Findings: Local pain, deformity, poor ROM & strength
        f. Imaging: x-ray (avulsion), MRI, US
        g. Treatment: Ice, stretching
19. Groin Strain
        a. Etiology: Tearing of Adductor muscle
        b. Mechanism: Powerful over stretch, abduct, external rotation common in soccer
        c. History: Pain which radiates along the medial thigh, inability to run, cut, start & stop
        d. Physical Findings: Pain over muscle group, increases with resistance, possible defect
        e. Imaging: MRI
        f. Treatment: Rest, ice, stretch
20. Piriformis Syndrome
        a. Etiology: Irritation to piriformis leading to sciatica
        b. Mechanism: Anatomical variance, tightness, overuse
        c. History: Cramping pain in buttock, tight hamstrings, tender piriformis, pain with sitting
        d. Physical Findings: pain stretching piriformis, weakness, sciatic tenderness, normal neuro
        e. Imaging: MRI (r/o other causes)
        f. Treatment: Rest, stretching, pain control, OMT
21. Tensor Fascia Latae Syndrome
        a. Etiology: Overuse tendinitis, Bursitis
        b. Mechanism: Running, after foot strike, hip 30o
        c. History: Pain during gait cycle.
        d. Physical Findings: Local pain, weakness of hip flexors, positive Ober’s
        e. Imaging: xray (r/o fracture)
        f. Treatment: Ice, pain control, stretching
    22. Case: Lisa is a 34 yo who has recently taken up running nad has increased her work out by 50%.
        She now complains of hip pain laterally worse when rising from a seated position. She states it
        gets better with movement but if her activity is prolonged it starts to hurt again.
           a. Greater Trochanteric Bursitis:
                     i. Pain that originates over the greater trochanteric bursa that may radiate the entire
                        length of the leg (knee and ankle but not foot)
                    ii. Etiology: Trauma to the bursa
                  iii. Mechanism: trauma acute or repetitive
                   iv. History: Localized pain, worse rising from chair , lessens with early movement then
                        worsens with extended movement; patients report night pain and cannot lay on
                        affected side ;increases with hip flex/ext
                    v. Physical Findings: Local pain, swelling at greater trochanter, pain cephalad to this
                        suggest tendinosis of gluteus medius tendon (Trendelenburg test is positive and
                        limp)
                   vi. Imaging: x-ray only to rule out other injury
                  vii. Treatment: Rest, Ice, NSAIDS, Injection, Correct biomechanics
                 viii. DDx:
                            1. Metastatic tumor – weight loss and B symptoms
                            2. Osteoarthritis – painful internal rotation
                            3. Sciatica – pain posteriorly or on top of foot
                            4. Snapping hip – clicking at site
                            5. Trochanteric fractire – limp persists with walking and postive Trendelnburg
                            6. Risks: lumbar spine disease, intraarticluar hip pathology, previous surgery
                                around lateral hip ( internal fixation device), RA
    23. Case: David is a 56 yo truck driver with a BMI of 40 (morbid obesity). He wears pants that are too
        small. He complains of pain and burning over the lateral thigh without any muscle weakness.
           a. Lateral femoral cutaneous nerve entrapment
                     i. Pain and burning (dysesthesia) or hypoesthesia over lateral thigh; they may
                        complain of groin pain and pain at SI joint;no motor involvement this is sensory
                        nerve
                    ii. Risks: obesity, tight clothing, surgery, trauma; nerve exits pelvis near ASIS
                  iii. Effects young muscular women who extend their hips, women with scoliosis and
                        joggers; rarely pathologic intra-abdominal/pelvic process
                   iv. Diagnostics – consider doing abdominal and pelvic exams to ensure no pelvic
                        pathology; no motor or DTR changes on exam; xrays of abdomen and pelvic, AP and
                        lateral of hip ,MRI
                    v. Differential:
                            1. Diabetes ,
                            2. osteoarhtritis – limited internal rotaion
Intra-abdominal tumor – weight loss, mass
                            3. Lumbar radiculopathy – quadriceps weakness, decreased reflexes
                            4. Trochanteric bursitis – tenderness over trochanter, Am stiffness
                   vi. Treatment: weight loss, steroid injection at site where nerve exits pelvis near
                        inguinal ligament, surgery for intractacble pain
    24. Review
           a. Know how to do the exam. Know special diagnostic Testing. Know pain patterns for ant,
               post, lateral pain in adult pt (and ones pointed out).
           b. For SP, look up the MSK ROS
OA
        Background
            o Most common type, aka DJD
            o Primarily affects cartilage, not bone (though there may be bone changes)
            o Slow, progressive degeneration
            o May be caused by persistent abnormal high loads on joint surfaces
            o Cartilage may crack, erode, and expose underlying bone
        Definitions
            o Cartilage: a hard but slippery coating on the end of each bone. Cartilage, breaks down and
                wears away in osteoarthritis.
            o Joint capsule: a tough membrane sac that holds all the bones and other joint parts
                together.
            o Synovium (sin-O-vee-um): a thin membrane inside the joint capsule.
            o Synovial fluid: a fluid that lubricates the joint and keeps the cartilage smooth and healthy.
            o Ligaments, tendons, and muscles: tissues that keep the bones stable and allow the joint to
                bend and move.
                     Ligaments are tough, cord-like tissues that connect one bone to another.
                     Tendons are tough, fibrous cords that connect muscles to bones.
                     Muscles are bundles of specialized cells that contract to produce movement when
                        stimulated by nerves.
        Cartilage: Key to Healthy Joints
            o Cart is 65-80% water; others are collagen, proteoglycans, and chondrocytes
            o Collagen (KAHL-uh-jen): a fibrous protein. Collagen is also the building block of skin,
                tendon, bone, and other connective tissues.
            o Proteoglycans (PRO-tee-uh-GLY-kanz): a combination of proteins and sugars. Strands of
                proteoglycans and collagen weave together and form a mesh-like tissue. This allows
                cartilage to flex and absorb physical shock.
            o Chondrocytes (KAHN-druh-sytz): cells that are found all through the cartilage. They mainly
                help cartilage stay healthy and grow. Sometimes, however, they release substances called
                enzymes that destroy collagen and other proteins.
        Case 1: 67yo male, increasing knee pain over last 6 mo, esp. last 2 months. Occasional Tylenol and
         motrin. No recent trauma. Occupation? Family Hx of OA? Pain intermittent and relieved with rest.
         Occasional swelling. Trouble stairs, Football player.
            o PE: Prehypertensive, normal pulse and temp
                     5’9” 223lbs
                     Varus angulation at knee
                     Joint margin tenderness bilaterally
                     Crepitus and painful ROM
                     Bouchard’s and Heberden’s nodes in hands
            o What now?
                     Radiograph. Show point space narrowing in medial compartment, subchondral
                        sclerosis, osteophytes
                     ** swelling, same pain/ROM problems  pseudogout
                     No specific lab test
                     Diagnostic joint aspiration. Rarely necessary except in joint pain is acutely worse or
                        are other signs of infection (fever, warmth, or redness). If signs of infection  tap it!
            o Discussion
                     Overweight, age, football player, occupation, family hx
            o Pathologic Basis
                Cartilage damage. Morpho changes in load-bearing areas of articular cartilage. In
                 early stages, cartilage is thicker than normal; with progression, cart softens,
                 integrity breach, vertical clefts (fibrillation). Deep cart ulcers, extending to bone.
                 Cartilage becomes hypocellular.
   OA of Knee may involve
        o Medial or lateral femorotibial compartment, patellofemoral
        o Palpation may reveal bony hypertrophy and tenderness
        o Small effusions, crepitus
        o Medial compartment: varus (bow-leg)
        o Lateral compartment: valgus (knock-knee)
   Joints




       o
   Hand OA
       o Heberden’s Nodes: bony enlargements of DIPJ, most common form of idiopathic OA
       o Bouchard’s nodes: PIP joints (Bouchard’s=body, closest to body)
       o May present acutely:
             Pain, redness, swelling, triggered by minor trauma




       o
       o Base of thumb: OA
   Treatment
       o Reduction of joint loading, orthotic
       o Exercise, PT
       o Drug Therapy
       o Intra-articular therapy (corticosteroids, hyaluronic)
       o Surgery
       o EXERCISE (strength, aerobic, ROM, agility, neck and back strength)
       o Meds
                 Simple analgesics
                 NSAIDs
                 Opioids
                 Homeopathic: Tumeric, MSM, Boswellia, Cetyl Myristoleate
                 Gemmotherapy: Pinus monatana, Ribes nigrum, Vitis vinifera
                 Intra-articular: corticosteroids (long-term: joint destructive), hyaluronic acid
                  (Synvisc, Hyalgan)
                 Glucosamine/chondroitin has been proven to work for some; fairly safe. Watch out
                  if you are diabetic, otherwise well-tolerated
   Risk Factors
       o Age (older than 65)
       o Female sex
       o No known race differences
       o Genetic: not understood, but correlated
       o Joint trauma/ repetitive stress
       o Obesity: HIGHEST CORRELATION
   Good cartilage/Bad cartilage





   Case 2: 63yo male, worked as manual laborer. One week hx of increasing back pain/stiffness. No
    recent trauma or new activities. Sx last no more than 2 days in past. Has taken Ibuprofen w/o
    resolution of pain
       o PE: vital signs are normal, decrease ROM in lower back. No tenderness in paraspinous
           muscles of back. Neurologic exam is non-focal
   Spine OA
       o DJD can involve apophyseal joint, discs, paraspinous ligaments (Spondylosis). Dx of spinal
           OA should be reserved for pts with involvement of apophyseal joint and not only disc
           degeneration. Sx include localized pain and stiffness. Nerve root compression by
           osteophyte blocking neural foramen, prolapse of degenerated disc, or subluxation of apop
           joint may cause radicular pain and motor weakness
   Definitions
       o Apophyseal joint- the joint around a bone that has no independent ossification
       o Spondylosis- ankylosis (stiffening) of the vertebrae (this term is often used very generally
           to refer to any degenerative back problem)
       o Spondylolysis- degeneration of the articulating part of the vertebrae (the classic OA
           change)
          o Spondylolisthesis- forward movement of the body of one of the lower vertebrae on the
              vertebrae below it
          o Spondylitis- inflammation of one or more of the vertebral bodies (infection [TB] or
              inflammatory disease [RA])
      Disc problems




          o


Bone and Joint Infections
      Osteomyelitis: Destruction of bone (infection)
          o Acute (~10 days)
                 Penetration, hematogenous spread, contiguous spread
          o Chronic
                 Associated with PVD (i.e., compromised blood supply)
          o Clinical Manifestations
                 Localized pain over affected bone; deep pain
                 Swelling and erythema from assoc. soft tissue infection
                 Pus draining from sinus tract
                 Constitutional symptoms (rare): fever (hematogenous spread), fatigue, malaise)
          o Causes: Gram-positive
                 S. aureus (60-80%), Strep Group A, B, C (10-20%), Staph epidermidis (10-15%)
          o Special Cases




               
          o Pathophys
          Bone resistant to infection
          S. aureus: skin colonizer, cause cellulitis and bacteremia, receptors for fibronetin
           and collagen
         Sites: age and mechanism dependent
                Vertebrae: hematogenous (discitis 1st – venous plexus) – older
                Long bones – younger kids. Distal femur, prox/distal tibia(Brodie’s abscess);
                   METAPHYSIS due to blood supply
                Contiguous: elderly and those with vascular compromise
                       o Feet (diabetics, PVD), pelvis and lower extremity (dubitus ulcer,
                          spinal cord injury)
o   Risk Factors
         Presence of foreign material (debris from wound, orthopedic hardware)
         Diabetes (microvascular disease, poor wound healing, neuropathy)
         Surgery
         Adjacent soft tissue infection
         PVD
         Sickle cell disease (devitalized bone)
         Congenital defects in phagocyte function
o   Osteomyelitis in Diabetes: Neuropathy leads to undiscovered foot ulceration
         Hx features
         Contiguous spread (direct inoculation)
         Sensorimotor neuropathy (predisposes to injury, educate for daily foot inspections)
                Lose distally first, using minofilament
         What organisms would you worry about? Staph, Strep, anaerobes
o   Establishing Dx
         Hx – risk factors
         PE
         Probe to bone (diabetic ulcers easier…)
         Blood work (CBC, ESR elevated, Blood culture (50% + in hematogenous)
         Aspirate cultures – normal in 25% cases
         Radiographs
o   What to do?
         Treat symptoms (oral antibiotics, then IV)
         Work-ups, imaging, etc.
o   DDx
         Cellulitis, gout/pseudogout, neuropathic joint, fx, aseptic bone infaction, sickle cell
           anemia
o   Radiographic Diagnosis
         Early xray normal (first signs, soft tissue swelling)
         Early positive due to osteoclastic activity at 14-21 days (usually periosteal
           elevation)
         By 28 days, 90% have abnormality (see it from day 1 on MRI)
         Osteolysis – destroy normal bone architecture
         Rarefaction – loss of bone density due to extremely active bone infection
         Bone fragments represent SEQUESTRUM
o   Advanced Imaging
         MRI: more sensitive, early
         CT: best with vertebral osteomyelitis
         US: can detect early changes – inexpensive
o   Tx: Antibiotics, 4-6 weeks parental antibiotics, immunization
                
        o Hx and PE – look for “red flags”
                 Low back pain: several weeks/months of bone pain; paraspinal muscle spasm,
                   percussion tenderness over spine; worse with valsalva, cough, strain
                 Nerve compromise: Parasthesia, bowel or bladder dysfunction, lower extremity
                   weakness
                 Draining wound rare
                 Fever 26-66% of pt
        o Most common site for contiguous-spread osteomyelitis: Foot
        o Hematogenous Spread
                 Most common: tibia, femur, humerus (kids); vertebra
                 Vertebral Osteomyelitis
                        LUMBAR spine most common, thoracic, cervical
                        Assoc with discitis and epidural abscess
                        Hematogenous: after minor trauma, elderly IV drug users, IV lines,
                           endocarditis pts at risk
                 Management Decisions
                        Establish Dx: vertebral biopsy “gold standard” necrotic bone, blood work
                           with cultures, radiographs/MRI
                        Tx: Parenteral antibiotics, empiric choice, most likely?
        o Chronic Osteomyelitis
                 Long clinical course, may develop pathologic fx, surgical tx
   Case: 45 year old is tearing down an old barn and steps on a nail. The nail breaks skin and “went
    deep.” Over the next 8 days he developed progressive pain, swelling and redness over the bottom
    of his foot. The pain became so severe that he couldn’t bear weight well and walks with a limp.
        o Most common cause of acute osteomyelitis: S. aureus
   Case: 14 year old is playing softball. She slides into second base and develops a “strawberry” that
    later becomes infected with cellulitis. It resolves with an OTC antibiotic cream. About 5 weeks
    later she presents with low back pain that is worse with any movement and coughing and
    straining at stool exacerbate the pain.
        o Hematogenous spread osteomyelitis
   Case: A 44 year old complains of severe knee pain beginning last night. It is associated with
    significant knee swelling, redness and warmth. He doesn’t want to bear weight on it this morning.
    Review of systems significant for fever to 102o last evening & night sweats.
        o Sx for infection: fast, localized, fever, night sweats
   JOINT INFECTION
        o DDx:
                 Infection
                 Gout/Pseudogout
                 Rheumatoid arthritis
                 Apetite related arthropathy
                 Reactive arthritis
                 SLE
        o Signs/Symptoms
                   Dx early is difficult, erythema, warm, swollen, loss of ROM
          o Pathogenesis
                   Direct penetration (trauma, surgery, bites, injection)
                   Extension into joint from adjacent infection
                   Hematogenous
          o Most common pathogen? S. aureus
                   Most septic: Staph
                   Gonococcal: college students
      Septic arthritis: knee, hip, shoulder, ankle, elbow
          o Etiology (special)
                   IV drug: Pseudomonas
                   Sexually active: N. gonorrhea
                   Endemic hikers – B. burgdorfii (lyme)
          o Dx
                   Joint aspiration
                   Fluid for culture (WBC>50,000, Gram stain, cultures)
                   Don’t go through skin infection to get to joint!!
                   Blood evaluation
                   LOOK AT FLUID
                           bacteria rapidly destroy joints with active infection; treat urgently
                           If you suspect infection urgent orthopedic referral is indicated


Rheumatoid Arthritis/ JRA
      RA: chronic systemic Autoimmune inflamm disease
          o Hallmark: synovitis affecting small joints of hands and feet symmetrically
          o Joints: MCP, PIP, knee, MTP, shoulder, ankle, cerv spine, hip, elbow, temperomandibular
          o Extra-articular involvement: skin, heart, lungs, eyes
          o Pathophys:
                  External trigger (infection, trauma) appears to be necessary to trigger an AI rxn in
                     genetically susceptible individuals
                  Synovial cell hyperplasia and endothelial cell activation are early events in the
                     pathologic process that progresses to uncontrolled inflammation.
                  Inflammation and proliferation of synovium (ie, pannus) leads to destruction of
                     tissues, including cartilage, bone, tendons, ligaments, and blood vessels.
                  Genetic factors and immune system abnormalities contribute to disease propagation
                  CD4 T cells, mononuclear phagocytes, fibroblasts, osteoclasts, and neutrophils have
                     major cellular roles in the pathophysiology of rheumatoid arthritis (RA).
                  B lymphocytes are involved with producion autoantibodies (ie, rheumatoid factors
                     [RFs]).
                  In patients with RA, there is documented abnormal production of numerous
                     cytokines, chemokines, and other inflammatory mediators that add to destructive
                     process
                  (eg, tumor necrosis factor alpha [TNF-alpha], interleukin [IL]–1, IL-6, transforming
                     growth factor beta [TGF-beta], IL-8, fibroblast growth factor [FGF], platelet-derived
                     growth factor [PDGF])
          o Etiology
                  Genetic factors account for 50% of the risk for developing RA.
                  Approximately 60% of US patients with rheumatoid arthritis carry a shared epitope
                     of the HLA-DR4 cluster, (eg, HLA-DR beta *0401, 0404, or 0405).
       HLA-DR1 (HLA-DR beta *0101) also carries this shared epitope and confers risk,
        particularly in certain southern European areas.
      Sequencing genes of families with RA suggest the presence of several susceptibility
        genes and several resistance genes.
      Juvenile idiopathic arthritis is a genetically complex trait in which multiple genes
        are important for disease onset and manifestations.
      The IL2RA/CD25 gene has been implicated as a juvenile idiopathic arthritis
        susceptibility locus, as has the VTCN1 gene.
      Infectious: Mycoplasma, EBV, rubella, etc.
      Hormones: sex hormones, WOMEN , recurrence post partum, reduced with oral
        contraceptives (hyperprolactinemia may be risk factor for RA)
      Immunologic factors
             All major immunologic elements play fundamental roles in the initiation,
                propagation, and maintenance of the autoimmune process of RA.
             T cells play a pivotal role in the initiation of RA, and the key player in this
                respect is assumed to be the T helper 1 (Th1) CD4 cells.
             Th1 cells produce IL-2 and interferon [IFN] gamma and subsequently
                activate macrophages and synovial fibroblasts.
             Macrophages and synovial fibroblasts are the main producers of the
                proinflammatory cytokines TNF-alpha and IL-1.
             Synovial macrophages and fibroblasts may become autonomous and lose
                responsiveness to T-cell activities in the course of the disease.
             B cells serve as antigen-presenting cells.
             B cells also produce numerous autoantibodies (eg, RF,to citrullinated
                proteins) and secrete cytokines
             RF+ anti-ccp?? Earlier antibodies are present, worse prognostic indicator
o Pathology
      Hyperactive/plastic synovial membrane produced  pannus tissue  invade
        cartilage and bone
      The major difference between RA and other forms of inflammatory arthritis, such as
        psoriatic arthritis, lies in the highly destructive potential of the RA synovial
        membrane and the local and systemic autoimmunity
      RA AI response  formation of immune complees that activate inflamm process to
        higher degree
o Epidemiology
      3/10,000 of population
      Women > men, equal after 65yo
      First-degree relatives have 2-3-fold increase; but not so high in monozygotic twins
        (genetics isn’t everything!)
o Prognosis
      Exacerbation/Remission
      40% disabled after 10 years
      The HLA-DRB1*04/04 genotype, a high serum titer of autoantibodies (eg RF, anti-
        cyclic citrullinated peptide [CCP]), extra-articular manifestations, a large number of
        involved joints, age younger than 30 years, female sex, and systemic symptoms all
        correlate with an unfavorable prognosis in terms of joint damage and disability.
      There is much worse prognosis of RA among patients with positive RF
      Other laboratory markers of a poor prognosis include early radiologic evidence of
        bony injury, persistent anemia of chronic disease, elevated levels of the C1q
        component of complement, and the presence of anti-CCP antibodies.
       The overall mortality rate in patients with RA is reportedly 2.5 times that of the
        general population.
      In those with severe articular and extra-articular disease, the mortality rate
        approaches that of patients with 3-vessel coronary disease or stage IV Hodgkin
        disease.
      Myocardial infarction, myocardial dysfunction, and asymptomatic pericardial
        effusions are common in patients with RA.
o TNF and Mortality
      Leading cause of mortality of RA is CVD followed by infection, resp disease, and
        malignancies
      Effects of concurrent immunosuppressive therapy may contribute to mortality in RA
      Studies confirmed that risk of mortality, serious infection, and malignancy is not
        increased in pts receiving anti-TNF therapy when pts have early RA and not treated
        with DMARDS and/or MTX
o Hx
      Insidious onset in most pts
      Begin with systemic features (fever, malaise, arthralgias, weakness) before
        appearance of joint inflammation and swelling
      Low grade fever, morning stiffness, weight loss common before inflamm
      Chronic RA most commonly results in progressive development of various degrees
        of joint destruction, deformity, and decline in functional status
o PE
      Small joints of hands/feet in symmetric distribution, inflamm, swelling, tender,
        warm, decreased ROM
             MCP>wrist>PIP>knee>MTP>shoulder>ankle>cerv spine>hip>elbow<TMJ
      Atrophy interosseous muscles is typical early finding  generalized atrophy
      Ulnar deviation, boutonniere, swan-neck deform, hammer toe, joint ankylosis
      Others: tenosynovitis, tendon rupture, OP, carpal tunnel
      Bout: Flex PIP, Extend DIP
      Swan-neck: Ext PIP, Flex DIP
      Trigger finger
      MCP Joints: volar subluxation, ulnar deviation
      Wrists: disrupt DRUJ with dorsal subluxation of ulna, rotation of carpus on distal
        radius with ulnarly translocated lunate. Entrapment neuropathy from synovitis
        about flexor tendons (of median nerve, carpal tunnel, ulnar nerve)
      Elbows and Shoulders: Elbow involvement is palpable synovial proliferation at
        Radiohumeral joint and flexion deformity (bursal involvement common as rheum.
        Nodules along extensor surface of elbow)
             RA shoulders tender, nocturnal pain, limited ROM; RC degeneration 2o to
                synovitis limit abduction and rotation. Glenohumarl damage leades to pain
                with motion and at rest  “frozen shoulder syndrome”
             Women more in shoulders and hips
             DON’T IMMOBILIZE
      Feet and Ankles: ankle joint uncommon w/o midfoot or MTP involvement
             Structural changes to midfoot and foot d/t to combo of chronic synovitis and
                weight-bearing
             Midfoot disease leads to loss of normal arch contour with flattening of the
                feet.
             Posterior tibialis tendon involvement or rupture may lead to subtalar
                subluxation, which results in eversion and migration of the talus laterally.
                        The MTP joints commonly become deformed over time.
                        The great toe typically develops hallux valgus (a bunion); subluxation of the
                         phalanx at the MTP joint of the other toes predominantly occurs dorsally.
                      The toes may exhibit compensatory flexion resulting in hammer toes .
                      The second and third metatarsal heads commonly protrude and may become
                         the primary weight-bearing surface at the MTP joints.
              Hips and Knees
                      Hips common (women>men)
                      Limited ROM, pain so can’t get up from chair
                      Knee effusions and synovial thickening common
                      Instable after loss of cartilage and weakening of ligaments
                      Deformity: valgus/varus
              Cervical Spine
                      Neck stiffness, pain, occipital headache (hx of RA more than 10 years)
                      AA joint major concern; compression syndromes, neuropathies, etc.
                      Neurologic involvement ranges from radicular pain to spinal cord lesions
                         that may result in weakness (including quadriparesis), sphincter dysfunction,
                         sensory deficits, and pathologic reflexes.
                      Transient ischemic attacks (TIAs) and cerebellar signs may reflect vertebral
                         artery impingement from cervical subluxation or basilar artery impingement
                         .
                      Tenosynovitis of the transverse ligament of C1 may lead to C1-C2 instability.
                             o Myelopathy secondary to rupture of the transverse ligament may lead
                                 to neurologic deficits.
                             o Radiculopathy is most common at the C2 root, although symptomatic
                                 subluxations may occur at any level.
       o Non-articular RA Involvement
              Cardiovascular morbidity and mortality are increased in patients with RA.
                      Myocardial infarction, myocardial dysfunction, and asymptomatic pericardial
                         effusions are common; symptomatic pericarditis and constrictive pericarditis
                         are rare.
              Myocarditis, coronary vasculitis, valvular disease, and conduction defects are
                 occasionally observed.
              Keratoconjunctivitis sicca is common in individuals with rheumatoid arthritis, and
                 this condition is often the initial manifestation of secondary Sjogren syndrome.
              Rheumatoid arthritis involvement of the lungs may involve pleural effusions,
                 interstitial fibrosis, nodules (Caplan syndrome), and bronchiolitis obliterans
                 organizing pneumonia.
              The liver is often affected in patients with Felty syndrome (ie, RA, splenomegaly, and
                 neutropenia).
              Rheumatoid nodules occur in approximately 25% of patients with RA, but they
                 occur in less than 10% of patients during the first year of the disease.
              Nerve entrapment is common, such as with the median nerve in carpal tunnel
                 syndrome and the cervical nerves with spine involvement.
              Vasculitic lesions, mononeuritis multiplex, and cervical myelopathy may cause
                 serious neurologic consequences.
              Vasculitic lesions of the skin may manifest as palpable purpura or skin ulceration
                 (eg, leg ulceration).
              Additionally, palmar erythema and pyoderma gangrenosum may be noted
   2010 RA Classification GUIDE
      o Patients who should be tested are those (1) with at least 1 joint with definite clinical
          synovitis and (2) whose synovitis is not better explained by another disease (eg, lupus,
          psoriatic arthritis, gout).
      o A score-based algorithm for RA based on 4 areas: joint involvement, serology test results,
          acute phase reactant test results, and patient self-reporting of signs/symptom duration.
      o A score of 6 of 10 or greater must be met for a classification of definitive RA.
      o Joint involvement consists of swelling or tenderness upon examination. The presence of
          synovitis may be confirmed on imaging studies.
      o Points are allocated as follows:
               1 large joint (ie, shoulders, elbows, hips, knees, ankles) = 0 points
               2-10 large joints = 1 point
               1-3 small joints (with or without involvement of large joints) (ie, MCP, PIP, second-
                  fifth MTP, thumb IP, and wrist joints ) = 2 points
               4-10 small joints (with or without involvement of large joints) = 3 points
               More than 10 joints (at least 1 small joint, plus any combination of large and
                  additional small joints or joints such as temporomandibular, acromioclavicular,
                  sternoclavicular, etc) = 5 points
      o At least 1 serology test result is needed for classification. Points are allocated as follows:
               Negative RF and negative ACPA = 0 points
               Low-positive RF or low-positive ACPA = 2 points
               High-positive RF or high-positive ACPA = 3 points
      o At least 1 test acute-phase reactant test result is needed.
               Normal CRP and normal ESR = 0 points
               Abnormal CRP or abnormal ESR = 1 point
      o Patient-reported duration of synovitis signs/symptoms of joints clinically involved
               Shorter than 6 weeks = 0 points
               6 weeks or longer = 1 point
   Measurement of Progression
      o Stage 1 (early RA): No destructive changes observed upon roentgenographic examination;
          radiographic evidence of osteoporosis is possible
      o Stage II (moderate progression): Radiographic evidence of periarticular osteoporosis, with
          or without slight subchondral bone destruction; slight cartilage destruction is possible;
          joint mobility is possibly limited, but no joint deformities are observed; adjacent muscle
          atrophy is present; extra-articular soft-tissue lesions (eg, nodules, tenosynovitis) are
          possible
      o Stage III (severe progression): Radiographic evidence of cartilage and bone destruction in
          addition to periarticular osteoporosis; joint deformity (eg, subluxation, ulnar deviation,
          hyperextension) without fibrous or bony ankylosis; muscle atrophy is extensive; extra-
          articular soft-tissue lesions (eg, nodules, tenosynovitis) are possible
      o Stage IV (terminal progression): Presence of fibrous or bony ankylosis, along with criteria
          of stage III
   Measure Functional Status
      o Class I - Completely able to perform usual activities of daily living
      o Class II - Able to perform usual self-care and vocational activities but limited in avocational
          activities
      o Class III - Able to perform usual self-care activities but limited in vocational and avocational
          activities
      o Class IV - Limited in ability to perform usual self-care, vocational, and avocational activities
   Measurement of Disease Remission
      o To be considered in remission, patients must meet at least 5 of the conditions below for at
          least 2 consecutive months:
               Duration of morning stiffness not exceeding 15 minutes
               No fatigue
               No joint pain
               No joint tenderness or pain with motion
               No soft-tissue swelling in joints or tendon sheaths
               An ESR level of less than 30 mm/h in a female or less than 20 mm/h in a male
               Predictors for remission of RA
   DDx (these don’t destruct the joint as much as RA)
       o Fibromyalgia
       o Lyme Disease
       o Myelodysplastic Syndrome
       o Osteoarthritis
       o Degenerative Joint Disease
       o Paraneoplastic Syndromes
       o Polychondritis
       o Gout and Pseudogout
       o Polymyalgia Rheumatica
       o Psoriatic Arthritis
       o Sarcoidosis
       o Sjogren Syndrome
       o Systemic Lupus Erythematosus
   Diagnostive Eval for RA
       o RF is an (Ig) M immunoglobulin antibody directed against the Fc fragment of IgG that is
          present in approximately 60-80% of patients with rheumatoid arthritis over the course of
          their disease
       o it is present in fewer than 40% of patients with early RA.
       o RF is not specific for RA, as it is also present in other connective tissue diseases, infections,
          and autoimmune disorders, as well as in 1-5% of healthy people.
       o Although antinuclear antibodies (ANA) are present in approximately 40% of patients with
          rheumatoid arthritis, test results for antibodies to most nuclear antigen subsets are
          negative.
       o Studies of anti-cyclic citrullinated protein (anti-CCP) antibodies suggest a sensitivity and
          specificity equal to or better than those of RF, with an increased frequency of positive
          results in early RA
       o The presence of both anti-CCP antibodies and RF is highly specific for RA.
       o The presence of anti-CCP antibodies, like that of RF, indicates a worse prognosis.
   Imaging
       o Xray first, MRI more sensitive (cervical subluxation, radiculopathy), Bone scan only for
          determining degree of inflammation
   Tx
       o Medication-based therapies comprise multiple classes of drugs, including NSAIDs,
          DMARDs, immunosuppressants, biologic response modifiers, and corticosteroids.
       o Early therapy with DMARDs has become the standard of care, as it not only retards disease
          progression more efficiently than later treatment, but it may also induce more remissions.
       o Delay of as little as 2-3 months in initiating joint-sparing therapy results in significant
          irreversible joint damage measured radiographically at 5 years.
       o Surgical treatments for RA include synovectomy, tenosynovectomy, tendon realignment,
          reconstructive surgery or arthroplasty, and arthrodesis.
       o Pharmacological Therapy
          DMARDS: gold salts (aurothiomalate, auranofin), D-penicillamine, chloroquine and
           hysroxychloroquine (HCQ), sulfasalazine (SSZ), methotrexate (MTX),
           azathioprine (AZP), Cyclosporin A
         MTX and SSZ are the most active compounds in terms of frequency of remissions
           and time to onset of action and provide the best risk-benefit ratios.
         MTX alone or in combination with other agents has become the standard of care for
           moderate to severe RA.
         Minocycline may act as a DMARD through its action as a matrix metalloproteinase
           inhibitor (MMPI).
         Leflunomide is the most recent addition to the xenobiotics and has activity similar
           to that of SSZ and MTX.
         Pts require 2-3months to achieve a full response to DMARDs
o   Toxicity
         Liver and bone marrow toxicity (MTX, SSZ, leflunomide, azathioprine, gold
           compounds, D-penicillamine)
         Renal toxicity (cyclosporin A, parenteral gold salts, D-penicillamine), pneumonitis
           (MTX)
         Allergic skin reactions (gold compounds, SSZ), autoimmunity (D-penicillamine, SSZ,
           minocycline)
         Infections (azathioprine, cyclosporin A)
o   Leflunomide
        
         Leflunomide blocks autoimmune antibodies and reduces inflammation.
         Leflunomide is a pyrimidine synthesis inhibitor, is extremely teratogenic and
           absolutely contraindicated in pregnancy.
         Its half-life is 14-15 days, but the active metabolite undergoes extensive
           enterohepatic circulation; thus, the drug takes up to 2 years to be undetectable in
           plasma.
o   MTX
         Methotrexate—a folic acid antagonist, is started at lower doses and increased to full
           doses within approximately 4-6 weeks.
         MTX is administered up to 25 mg once a week.
         Approximately 1% of patients develop pneumonitis while taking methotrexate
           (MTX).
         MTX is contraindicated in pregnancy because it is an abortifacient and has
           teratogenic effects, including craniofacial abnormalities, limb defects, and CNS
           defects such as anencephaly, hydrocephaly, and meningomyelopathy, especially
           with first-trimester exposure
o   Bio agents
         Agents that block TNF-alpha and IL-1 induced cytokines or their effects are widely
           utilized in treatment of RA.
         The TNF blockers, which bind TNF and thus prevent its interaction with its
           receptors, include etanercept, infliximab, and adalimumab:
         infliximab binds to cells that express membrane TNF,
         etanercept binds lymphotoxin (formerly termed TNF-beta) in addition to soluble
           TNF-alpha
         Golimumab, a new human anti-TNF-alpha monoclonal antibody, inhibits TNF-alpha
           bioactivity, thereby modulating immune activity in patients with RA
         Rituximab, a monoclonal antibody against CD20 protein, has been shown to be
           effective in reducing the signs and symptoms in adult patients with moderately to
          severely active RA who have had an inadequate response to therapy with one or
          more TNF antagonist
        Most often used in combination with MTX.
o   Immunomodulators
        Anakinra (IL-1 receptor antagonist ) [IL-1ra].
        IL-1ra occupies the IL-1 receptor without triggering it (blocking) and prevents
          receptor binding of IL-1.
               In clinical trials, a significant response was observed in approximately 40%
                  of patients with RA.
        Abatacept is a selective co-stimulation modulator that inhibits T-cell activation by
          binding to CD80 and CD86, thereby blocking their interaction with CD28.
               CD28 interaction provides the signal needed for full T-cell activation
                  implicated in rheumatoid RA pathogenesis.
        Tocilizumab (Actemra) is an interleukin 6 (IL-6) receptor inhibitor.
               Indicated for moderate-to-severe active RA in adults who have had an
                  inadequate response to one or more TNF-antagonist therapies.
               It may be used alone or in combination with methotrexate or other disease-
                  modifying antirheumatic drugs.
o   Adverse Effects of Biolgoical
        Adverse effects associated with the biologic agents include the generation of
          antibodies against the compounds, emergence of antinuclear antibodies, occasional
          drug-induced lupus like syndromes, and infections (including tuberculosis).
        Rarely, demyelinating disorders and bone marrow suppression occur.
        Acute and chronic infections, demyelinating disorders, class 3 and 4 heart failure,
          and recent malignancies are contraindications for TNF blockers.
        Thoroughly searching for latent tuberculosis using chest radiography and/or
          purified protein derivative (PPD) testing is recommended before these agents are
          started.
        Patients taking anti-TNF agents must avoid live-virus vaccines
o   NSAIDS and Steroids
        Glucocorticoids are potent anti-inflammatory drugs used in patients with RA to until
          DMARDs are effective and for control of acute flares.
        Significant adverse effects are associated with long-term steroid use.
        Heart failure, hypertension, diabetes, osteoporosis, ASCAD, etc.
        NSAIDs interfere with prostaglandin synthesis through inhibition of the enzyme
          cyclooxygenase (COX), thus reducing swelling and pain.
        They do not retard joint destruction and, therefore, are not sufficient to treat RA
          alone.
        Traditional NSAIDs inhibit both COX-1 and COX-2.
        Celecoxib, a COX-2 inhibitor, has significant preference for COX-2 over COX-1,
          providing more protection for the G.I. system.
o   Combo Therapy
        Several combinations have proved successful and without unexpected added risks;
          these combinations usually include MTX (ie, MTX plus SSZ plus an antimalarial, MTX
          plus leflunomide, or MTX plus biologic agents)
        Although the combination is not commonly used, cyclosporine with MTX results in
          greater clinical improvement than MTX alone.
        Triple therapy with MTX, SSZ, and HCQ may provide substantially greater clinical
          improvement than MTX alone or SSZ plus HCQ
        In combination with infliximab, MTX provides a superior response to monotherapy.
                 In combination with rituximab, MTX provides a superior response to monotherapy.
                 In combination with etanercept, MTX provides a higher rate of meaningful clinical
                  response.
   Juvenile RA
       o Most common form of childhood arthritis; different from Adult RA!
       o ACR define it as less than 16yo, duration of greater than 6 weeks
       o Subtypes
                Polyarticular (over 5 joints)
                Pauciarticular (1-4 joints)
                Systemic (constitutional symptoms and joint involvement)
       o Other types (juvenile akylosing spondy, psoriatic arth) are spondyloarthropathies
   ILAR
       o Systemic-onset JIA
       o Persistent or extended oligoarthritis
       o Rheumatoid factor (RF)–positive polyarthritis
       o RF-negative polyarthritis
       o Psoriatic JIA
       o Enthesitis-related arthritis
       o Undifferentiated - The disease does not meet criteria for any of the other subgroups, or it
           meets more than 1 criterion (and therefore could be classified in a number of subgroups).
   EULAR
       o The EULAR proposed the term juvenile chronic arthritis (JCA) for the heterogeneous group
           of disorders that manifest as juvenile arthritis.
       o The diagnosis requires that the arthritis begins before age 16 years and lasts for at least 3
           months.
       o The EULAR criteria for JCA recognize the following subtypes, based on characteristics at
           onset:
       o Pauciarticular (1-4 joints)
       o Polyarticular (≥5 joints)
       o Presence of RF
       o Systemic onset with characteristic features
       o Positivity for rheumatoid factor
       o Juvenile ankylosing spondylitis
       o Juvenile psoriatic arthritis
   Juvenile Idiopathic Arthritis
       o JRA and the new nomenclature, Juvenile Idiopathic Arthritis (JIA), represents a group of
           disorders that share the clinical manifestation of chronic joint inflammation that are
           genetically complex.
       o The IL2RA/CD25 gene has been implicated as a JIA susceptibility locus, as has the VTCN1
           gene.
       o Humoral and cell-mediated immunity are also involved in the pathogenesis of JIA.
       o T lymphocytes have a central role, releasing proinflammatory cytokines and favoring a
           type-1 helper T-lymphocyte response.
       o humoral immune system exhibits increased presence of autoantibodies (especially
           antinuclear antibodies), increased serum immunoglobulins, circulating immune complexes,
           and complement activation.
       o Chronic inflammation of synovium is characterized by B-lymphocyte infiltration and
           expansion.
       o Macrophages and T-cell invasion are associated with the release of cytokines, which evoke
           synoviocyte proliferation.
       o Approximately 300,000 children in the United States are estimated to have some type of
           arthritis.
       o The incidence rate estimates for JIA range from 4-14 cases per 100,000 children annually;
           for JRA, the prevalence has ranged from 1.6 to 86.1 cases per 100,000.
       o Disease-associated mortality for JIA is difficult to quantify, but it is estimated to be less than
           1% in Europe and less than 0.5% in North America
   JRA Distribution
       o The approximate frequencies of the various forms of JRA are as follows:
       o Oligoarticular - 30%
       o Polyarticular RF negative - 20%
       o Polyarticular RF positive – 5%
       o Systemic-onset – 5%
       o Psoriatic - 5%
       o Enthesitis Related – 25%
       o Undifferentiated – 10%
   Sex and Age distribution
       o Girls with an oligoarticular onset outnumber boys by a ratio of 3:1.
       o Polyarticular onset, girls outnumber boys by 2.8:1
       o With uveitis, the ratio of girls to boys is 5-6.6:1
       o Systemic-onset occurs with equal frequency in boys and girls.
       o Boys outnumber girls with enthesitis-related arthritis
       o Although JIA is defined as arthritis beginning before age 16 years, the age at onset is often
           much lower, with the highest frequency occurring in children aged 1-3 years.
       o RF-positive disease is more common in adolescents.
       o The usual age of onset of enthesitis-related arthritis is 10-12 years (at attachment of
           bone).
   Prognosis
       o Those with polyarticular disease, may have problems with active disease throughout
           adulthood, with sustained remission attained in a minority of patients.
       o Most children with oligoarticular disease demonstrate eventual permanent remission,
           although a small number progress to persisting polyarticular disease.
       o Compared with adults with RF-positive rheumatoid arthritis, however, children are at less
           risk for rheumatoid lung involvement and vasculitis.
   Hx
       o Disease onset is either insidious or abrupt, with morning stiffness or gelling phenomenon
           (ie, stiffness after long periods of sitting or inactivity) being a frequent complaint and
           arthralgia occurring during the day.
       o A morning limp that improves with time may be noted, and a toddler may no longer stand
           in the crib in the morning or after naps.
       o Children often stop using joints normally (eg, develop contractures of joints, decreased
           wrist range, limp) rather than complain of pain.
       o Up to a quarter of children with oligoarticular JIA have no pain.
       o Systemic-onset JIA is characterized by spiking fevers, typically occurring once or twice each
           day, at about the same time of day.
       o Systemic-onset JIA is usually accompanied by an evanescent rash (lasting a few hours),
           which is typically nonpruritic, macular, and salmon colored on the trunk and extremities.
       o Enthesitis-related arthritis frequently presents as evening and post-exercise pain.
           Attention should be given to buttock pain and back pain that improves with activity
           (inflammatory back pain).
        o Children with psoriatic arthritis may have typical psoriasis but dermatological
           manifestations may be subtle
   Diagnostic for JRA:
        o No diagnostic serologic tests for JIA are recognized, aside from rheumatoid factor assay for
           subclassification of polyarticular disease.
                antinuclear antibody and HLA-B27 assays, may help further define diagnosis and
                   risk of complications.
        o As many as 70% of children with oligoarticular JIA have positive ANA assays.
                A positive ANA should also raise suspicion of systemic lupus erythematosus(SLE).
        o A positive ANA is a marker for increased risk of anterior uveitis.
                Children younger than 6 years at arthritis onset with a positive ANA finding are in
                   the highest risk category for development of uveitis and need slit lamp screening
                   every 3-4 months.
        o MRI provides the most sensitive radiologic indicator of disease activity because it can
           depict synovial hypertrophy, define soft tissue swelling, and demonstrate detail of the
           status of articular cartilage and overall joint integrity
        o Arthritis presence is key to diagnosis and is defined as either intra-articular swelling on
           examination or as limitation of joint motion in association with pain, warmth, or erythema
           of the joint.
        o With synovitis, and its synovial proliferation and an increase in joint volume, the joint is
           held in a position of maximum comfort.
                Limbs with synovitis are generally held in flexion.
        o The hip is held in an attitude of flexion, abduction, and external rotation.
        o The wrist and knee are most commonly is in flexion.
   Systemic Arthritis
        o The child appears systemically ill
        o Arthralgia is often present and pt. may have generalized myalgia
        o Evanescent, salmon-pink, macular rash (often linear) is found, predominantly on the trunk
           and the extremities; this rash, is associated with fever spikes
        o Hepatosplenomegaly is often present
        o Lymphadenopathy is sometimes present, especially the axillary lymph nodes
        o Serositis, including pleural and pericardial effusions, may be present.
        o Chest pain or shortness of breath may be a sign of pericarditis or pleuritis
        o Friction rub may occur in pericarditis but can be absent with a large pericardial effusion
        o S3, basilar rales, and hepatomegaly suggestive of heart failure may rarely be observed when
           myocarditis occurs in individuals with systemic-onset JIA
   Oligoarticular JIA
        o Large, weight-bearing joints, such as the knees and ankles, are typically affected.
        o Child appears well except for joint pain or limp
        o Involvement small joints in the hands is atypical and suggests eventual development of
           polyarticular JIA or psoriatic arthritis.
        o Dactylitis, or diffuse tenosynovitis of a finger or toe, also called a “sausage digit,” is more
           typical of psoriatic arthritis or enthesitis-related arthritis.
        o Anterior uveitis is present in as many as 20% of children with oligoarticular and
           polyarticular JIA, especially those who are antinuclear antibody (ANA) positive.
   Polyarticular JIA
        o In polyarticular juvenile idiopathic arthritis, 5 or more joints are affected in the first 6
           months after disease onset, weight-bearing joints are often affected, rheumatoid nodules
           may be seen in patients with RF-positive disease, and symmetrical involvement of small
           joints in the hands is often found
       o Arthritis of the cervical spine and can lead to subluxation, typically of the C2 vertebra on
            C3.
       o Arthritis of the temporal-mandibular joint (TMJ) may lead to micrognathia
   Psoriatic Arthritis
       o Onset of arthritis, usually mild, precedes that of psoriasis in approximately half of children.
       o Characteristics of psoriatic arthritis include the following:
       o Monoarticular arthritis (50% of children)
       o DIP joint involvement (50%)
       o Tenosynovitis (30%)
       o Nail involvement(71%) - pitting is the most common but least specific finding
       o Disordered bone growth with resultant shortening (47%)
       o Sacroiliitis (28%)
   Enthesitis- Related Arthritis
       o Enthesitis-related arthritis, or pediatric spondyloarthropathy, is characterized by periods
            of inflammation of tendons and ligaments, at the area of insertion into bone (entheses).
       o Pain and tenderness is the most common manifestation, but swelling may also be seen.
       o The initial manifestations involve mainly the peripheral joints (eg, dactylitis) with
            asymmetric oligoarticular arthritis of the lower limbs
       o Axial involvement (eg, sacroiliitis) tends to appear later in the disease course
       o Diagnostic criteria are the presence of both arthritis and enthesitis, or the presence of
            arthritis or enthesitis along with any 2 of the following 5 manifestations[15] :
                 Sacroiliac tenderness and/or inflammatory lumbosacral pain
                 Positive human leukocyte antigen B27 (HLA-B27) test
                 Onset of arthritis in a male 6 years old or older
                 Acute symptomatic anterior uveitis
                 Presence in a first-degree relative of ankylosing spondylitis, enthesitis-related
                    arthritis, inflammatory bowel disease with sacroiliitis, reactive arthritis, or acute
                    anterior uveitis
   Tx of JIA
       o Management may include one or all of the following areas:
       o Pharmacologic management consisting of nonsteroidal anti-inflammatory drugs (NSAIDs),
            disease-modifying antirheumatic drugs (DMARDs), biologic agents, and intra-articular and
            oral steroids
       o Psychosocial factors, including counseling for patients, parents, teachers
       o Nutrition, particularly to address anemia and generalized osteoporosis; often microcytic,
            anemia is refractive to treatment with iron
       o Physical therapy to relieve pain and to address range of motion, muscle strengthening,
            activities of daily living, and conditioning exercises
       o Occupational therapy, including joint protection, a program to relieve pain, range of
            motion, and attention to activities of daily living
   Treatment Regimens
       o In 2011, the American College of Rheumatology issued recommendations for the treatment
            of JIA based on 5 treatment groups.
       o A history of arthritis in 4 or fewer joints
       o A history of arthritis in 5 or more joints
       o Active sacroiliac arthritis
       o Systemic arthritis without active arthritis
       o Systemic arthritis with active arthritis
   Hx of Arthritis in 4 or fewer joints
       o NSAIDs alone may be adequate for patients with involvement of a single joint and other
           indications of low disease activity (eg, normal inflammatory marker levels); response
           should be evident within 2 months.
       o Intra-articular injections of triamcinolone can be used for any joint involved with active
           arthritis, and should provide clinical relief for at least 4 months
       o Methotrexate is recommended as initial treatment for patients in this treatment group who
           have high disease activity and features indicating poor prognosis.
       o In patients with enthesitis-related JIA, sulfasalazine rather than methotrexate is
           recommended for patients who have an inadequate response to joint injection or an
           adequate trial of NSAIDs.
       o Patients who fail to respond adequately to joint injections and to 3-6 months of
           methotrexate are candidates for TNF-alpha treatment.
   5 or more joints
       o This group includes patients with the ILAR categories of extended oligoarthritis,
           rheumatoid factor (RF) negative and RF-positive polyarthritis, psoriatic arthritis,
           enthesitis-related arthritis, and undifferentiated arthritis.
       o Treatment in this group places less emphasis on initial NSAIDs: treatment may start with
           methotrexate. Leflunomide may be used as an alternative to methotrexate.
       o Escalation to a TNF-alpha inhibitor follows if 3-6 months (depending on disease
           characteristics and severity) of methotrexate or leflunomide provides inadequate control.
       o If these agents prove inadequate, patients may be started on rituximab; this agent may be
           most appropriate in patients with RF-positive polyarticular JIA
   Active SI Arthritis
       o Use of a TNF-alpha inhibitor is recommended more readily for patients in this group.
       o A TNF-alpha inhibitor may be started after failure of an adequate trial of NSAIDs or after 3-
           6 months (depending on disease characteristics and severity) of methotrexate or
           sulfasalazine proves inadequate.
   Systemic Arthritis with Active Systemic Features and w/o active arthritis
       o Patients with high systemic disease activity (eg, significant serositis) may be started on
           steroids as a first step.
       o Patients who sustain or develop active fever while on systemic steroid therapy can be
           started on anakinra.
                This agent may be a first choice in patients who have had significant active systemic
                   disease for at least 6 months.
       o In April 2011, the US Food and Drug Administration (FDA) granted orphan drug status to
           the interleukin-6 (IL-6) inhibitor tocilizumab (Actemra).
                This agent is approved for use as monotherapy or in combination with methotrexate
                   for the treatment of active systemic JIA in children age 2 years and older.
   Systemic Arth with active arth and w/o active systemic features
       o NSAID therapy, with intra-articular joint injections as needed, may be adequate for patients
           with low disease activity who do not have hip involvement or radiographic signs of joint
           damage.
       o After up to 1 month, methotrexate is added for patients with any degree of disease severity
           who continue to have active arthritis.
       o After 3 months of methotrexate therapy, the next step in escalation is to anakinra or a TNF-
           alpha inhibitor.
       o Patients who show inadequate response to TNF-alpha inhibitor treatment can be started
           on abatacept.
   Tx of Macrophage Activation Syndrome
       o Macrophage activation syndrome (MAS) is a rare but important complication of systemic-
           onset JIA in which numbers of all 3 bloodlines become rapidly decreased.
           Hypofibrinogenemia, thrombocytopenia, and elevated aspartate aminotransferase levels
           are hallmarks.
       o MAS often responds to cyclosporin A, and some case reports have detailed a response to
           anakinra.
       o Treatment of MAS is a medical emergency and should be performed by physicians familiar
           with this complication.
   Tx of Uveitis
       o Patients are typically young girls who have positive levels of ANA and RF.
       o Treatment with topical corticosteroid medication and with mydriatic agents (to prevent
           closed-angle glaucoma) often can prevent progression of disease and development of
           calcium deposition in the lens (band keratopathy) and adhesions of the iris to the lens
           (posterior synechiae), in which an irregular pupillary margin develops.
       o Immunosuppressive agents, such as methotrexate or cyclosporine, may help control
           chronic uveitis.
       o Infliximab can be effective in some patients who are resistant to immunosuppressive
           agents.
   Abatacept (Orencia)
       o Abatacept is a selective costimulation modulator that inhibits T-cell activation by binding
           to CD80 and CED86, thereby blocking CD28 interaction.
       o It is indicated for reducing signs and symptoms of RA, slowing progression of structural
           damage and improving physical function in adults with moderate-to-severe RA who have
           inadequate response to DMARDs, MTX, or TNF antagonists.
                It is not recommended for concomitant use with anakinra because of insufficient
                  experience.
       o The pediatric dosage is not established for patients younger than 6 years.
   Adalimumab (Humira)
       o Adalimumab is a recombinant human IgG1 monoclonal antibody that is specific for human
           TNF.
       o The pediatric dosage has not been established for patients younger than 4 years
       o Patients older than 4 years and more than 15 kg but less than 30 kg, the dosage is 20 mg SC
           q2wk, and for patients older than 4 years and heavier than 30 kg, the dosage is 40 mg SC
           q2wk.
   Etanercept (Enbrel)
       o Etanercept acts by binding and inhibiting TNF, a cytokine that contributes to inflammatory
           and immune response.
       o The pediatric dosage is not established for patients younger than 4 years.
       o For patients 4-17 years, the dosage is 0.4 mg/kg SC 2 times weekly (administered at least
           72-96 h apart), not to exceed 25 mg/dose.
       o For patients older than 17 years, the dosage is administered as in adults.
   Anakinra (Kineret)
       o Anakinra competitively and selectively inhibits IL-1 binding to type I receptor (IL-1RI).
       o It is indicated for rheumatoid arthritis in patients who have failed 1 or more DMARDs. The
           dose should be administered at approximately the same time every day.
       o While the adult dosage is 100 mg SC qd; the pediatric dosage has not been established.
   Tocilizumab (Actemra)
       o Tocilizumab is an IL-6 receptor antagonist that is indicated for systemic JIA.
       o The safety and efficacy of tocilizumab has not been established in patients < 2 years.
          o For patients 2 years or older and < 30 kg, the dose is 12 mg/kg IV q2wk; for those ≥30 kg,
            the dose is 8 mg/kg IV q2wk.


Polymyositis, Dermatomyositis and Polyarthritis Rheumaticia
      Idiopathic Inflamm Myopathies: Bohan and Peter Classification
           o I - Primary idiopathic polymyositis
           o II - Primary idiopathic dermatomyositis
           o III - Polymyositis or dermatomyositis associated with malignancy
           o IV - Childhood polymyositis or dermatomyositis
           o V - Polymyositis or dermatomyositis associated with another connective-tissue disease
           o VI - Inclusion body myositis (stroke, blindness)
           o VII - Miscellaneous (eg, eosinophilic myositis, myositis ossificans, focal myositis, giant cell
              myositis)
      Polymyositis
           o Polymyositis is an idiopathic, immune mediated, inflammatory myopathy that causes
              symmetrical, proximal muscle weakness; elevated skeletal muscle enzyme levels; and
              characteristic electromyography (EMG) and muscle biopsy findings.
           o It is a T-cell–mediated cytotoxic process directed against muscle antigens.
                   CD8 T cells, along with macrophages, initially surround healthy nonnecrotic muscle
                      fibers and eventually invade and destroy them
           o It may occur alone or in association with viral infections, malignancies, or often in
              conjunction with other connective-tissue disorders.
           o The viruses appear to damage the vascular endothelium, release cytokines, which induce
              abnormal expression of the major histocompatibility complex (MHC) and render the
              muscle susceptible to destruction.
           o The human retroviruses human immunodeficiency virus (HIV) and human T-cell
              lymphotrophic virus type I (HTLV-I), the simian retroviruses, and coxsackievirus B have
              been etiologically connected with the disease
      Autoimmune response
           o An autoimmune response to nuclear and cytoplasmic autoantigens is detected in 60-80%
              of patients with polymyositis and dermatomyositis.
           o Myositis Associated Antibodies (MAA) are also associated with other autoimmune
              diseases), and Myositis specific antibodies (MSA) are unique to myositis.
           o The MSAs are found in approximately 40% of patients with polymyositis or
              dermatomyositis, whereas MAAs are found in 20-50% of these patients.
      Myositis-Specific antibodies
           o MSA targets include 3 distinct groups of proteins: aminoacyl–transfer ribonucleic acid
              (tRNA) synthetases (anti-Jo-1), nuclear Mi-2 protein, and components of the signal-
              recognition particle (SRP)
           o Anti-histidyl-tRNA synthetase (Jo-1) is most common (20-30%) identified antibody and
              recognized to be specific for polymyositis.
           o The presence of anti-Jo-1 antibodies defines a distinct group of polymyositis patients with
              interstitial lung disease, arthritis, and fevers.
           o Mi-2 autoantibidies are specific serologic markers of dermatomyositis.
                   Detected in about 20% of patients with myositis they are associated with relatively
                      acute onset, a good prognosis, and a good response to therapy.
           o Patients with anti-SRP antibodies have acute polymyositis with cardiac involvement, a poor
              prognosis, and a poor response to therapy.
        o The most important antigenic targets of the MAA are the PM/Scl nucleolar antigen, the
           nuclear Ku antigen, the small nuclear ribonucleoproteins (snRNP), and the cytoplasmic
           ribonucleoproteins (RoRNP).
        o Anti-PM/Scl autoantibodies are generally found in patients affected by polymyositis
           overlapping with scleroderma.
        o Anti-Ku antibodies are found in patients with myositis overlapping with other connective
           tissue diseases such as RA.
        o Antibodies against snRNP are frequently found in patients with myositis and in patients
           with connective tissue–disease overlap syndrome.
        o Antibodies toward components of the RoRNP complex are almost exclusively found in
           patients with Sjögren syndrome and systemic lupus erythematosus (SLE).
   Risk Factors
        o Increased myositis is associated with human leukocyte antigen (HLA) haplotypes A1, B8,
           and DR3.
        o Environmental triggers include the following:
                Coxsackievirus B1
                HIV
                HTLV-1
                Hepatitis B
                Influenza
                Echovirus
                Adenovirus
        o Several drugs induce an immune-mediated myopathy or myositis.
                D-penicillamine, hydralazine, procainamide, phenytoin (precipitation of inflamm
                   reactions in autoimmune disease), and angiotensin-converting enzyme (ACE)
                   inhibitors have been associated with this type of inflammatory myopathy.
                Statins occasionally cause a different varrient of severe muscle inflammation and
                   rhabdomyolysis.
   Epi
        o Idiopathic inflammatory myopathies are rare diseases, with an incidence in the United
           States that ranges from 0.5-8.4 cases per million population.
        o Polymyositis is more common in the United States within the black population, with the
           estimated black-to-white incidences for polymyositis and dermatomyositis being 5:1 and
           3:1, respectively.
        o Polymyositis and dermatomyositis are more common in women than in men (2:1 ratio)
        o Inclusion body myositis is twice as common in men.
        o Polymyositis usually affects adults aged 45-60 years.
                Polymyositis rarely affects children.
        o Dermatomyositis is primarily a disease of adults, it also is observed in children, usually
           those aged 5-14 years.
        o Eighty percent of patients with inclusion body myositis are older than 50 years at onset.
   Prognosis
        o Five-year survival rates have been estimated at more than 80%.
        o Mortality is most often related to associated malignancy or pulmonary complications;
           however, elderly patients with cardiac involvement or dysphagia also have a higher
           mortality rate.
        o Poor prognostic factors include the following:
                Advanced age
                Female sex
                African American race
                Interstitial lung disease
               Presence of anti-Jo-1 (lung disease) and anti-SRP antibodies (severe muscle disease,
                cardiac involvement)
               Associated malignancy
               Dysphagia, dysphonia
               Cardiac and pulmonary involvement
       o Complications of polymyositis may include the following:
               Interstitial lung disease, pneumoniae,
               Cardiac muscle disease
               Arrhythmias
               Pericarditis
               Dysphagia and reflux
               Malabsorption
               Carcinoma - Especially in the breast and lung
               None erosive arthralgia
   Hx and PE
       o Symptoms of polymyositis gradually develop over a period of 3-6 months.
               No associated rash occurs before the onset of muscle disease unlike
                dermatomyositis.
               Symmetrical, proximal muscle weakness with insidious onset
                     Difficulty kneeling, climbing or descending stairs, stepping onto a curb,
                        raising arms, lifting objects, combing hair, and arising from a seated position
                     Weak neck extensors cause difficulty holding the head up
                     Involvement of pelvic girdle usually greater than upper body weakness
               Myalgias occur in fewer than 30% of patients
               Dysphagia (30%), aspiration, dysphonia
               Arthralgias occasionally ( not destructive )
               Cardiac involvement may cause symptoms of pericarditis or cardiomyopathy
       o History:
               Polymyositis has been associated with other connective-tissue diseases, including
                the following:
               Systemic lupus erythematosus
               Rheumatoid arthritis
               Mixed connective-tissue disease
               Sjögren syndrome
               Scleroderma
       o PE: mostly large muscle groups (ocular spared; DTR normal)
               Muscles of the trunk, shoulders, hips, upper arms, and thighs are usually involved.
               Ocular muscles remain normal even in advanced, untreated cases.
               Facial muscles remain normal except in rare advanced cases.
               The pharyngeal and neck flexor muscles are often involved, causing dysphagia and
                difficulty in holding up the head.
               In advanced cases and rarely in acute cases, respiratory muscles are affected.
                Dysphonia with nasal speech may be noted. Lung examination findings may include
                evidence of interstitial lung disease, such as dry inspiratory crackles in the lung
                bases (“Velcro”).
               Sensory examination findings are normal.
               The tendon reflexes are preserved, but they may be absent in severely weakened or
                atrophied muscles.
               Primary cardiac abnormalities due to myocarditis may be present in a few patients.
       o Differentials
                 Conditions to consider in the differential diagnosis of polymyositis include the
                  following:
                       Hypokalemia (and muscle weakness)
                       Myopathies
                       Muscular dystrophy
                       Myasthenia gravis
                       Overlap connective-tissue diseases
                Drug-induced myopathy may result from the following:
                       Antimalarials
                       Clofibrate
                       Colchicine
                       Ketoconazole and other azole antifungal agents
                       Statin/3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase
                         inhibitors (CK gets very high)
                       D-penicillamine
                       Vincristine
                       Zidovudine (AZT)
                Differentials
                       Amyotrophic Lateral Sclerosis (ALS) in Physical Medicine and Rehabilitation
                       Fibromyalgia
                       Hyperthyroidism
                       Hypothyroidism
                       Polymyalgia Rheumatica
                       Rheumatoid Arthritis
                       Sarcoidosis
                       Systemic Lupus Erythematosus
   Eval and Work Up
       o Leukocytosis is present in more than 50% of patients
       o Erythrocyte sedimentation rate or C-reactive protein level - Elevated in 50% of patients
           with polymyositis
       o Myoglobinuria
       o Positive rheumatoid factor results - Found in more than 50% of patients
       o Serum creatine kinase (CK) levels are usually elevated in persons with polymyositis,
           ranging from 5-50 times the reference range.
       o Aldolase - usually only the CK and aldolase levels are determined
       o Only diagnostic: Sed rate and EMG
       o Antinuclear antibody (ANA) assay - Positive in 30-40% of patients with polymyositis and in
           only 15% of patients with inclusion body myositis
       o Antisynthetase antibodies (anti-Jo-1 antibodies) - manifest as idiopathic inflammatory
           myopathy, interstitial lung disease, arthritis, Raynaud phenomenon, fever, and/or
           mechanic’s hands
       o Signal-recognition particle (SRP) antibodies - Approximately 4% of patients with
           polymyositis.
       o Associated with acute onset of severe weakness, increased incidence of cardiac
           involvement, and higher mortality rates
       o Muscle-imaging techniques such as magnetic resonance imaging (MRI) and
           ultrasonography may document and localize the extent of muscle involvement.
       o MRI scans show signal intensity abnormalities of muscle due to inflammation, edema, or
           scarring.
       o Perform age-appropriate evaluation for malignancy (breast, colon, lymphoma, etc.)
       o Electromyographic findings are abnormal in almost all patients (90%) with polymyositis.
       o Muscle biopsy shows muscle fibers in varying stages of inflammation, necrosis, and
          regeneration.
       o focal endomysial infiltration by mononuclear cells, capillary obliteration, endothelial cell
          damage, and increased amounts of connective tissue.
       o Perifascicular atrophy or prominent perivascular infiltrates are not present, and the
          blood vessels are normal unlike dermatomyositis.***** ON TEST FOR SURE
   Treatment
       o Prednisone is the first-line treatment of choice for polymyositis.
               Usual dose is 1 mg/kg/day, either as a single or divided dose. This high dose is
                 usually continued for 4-8 weeks, until the CK level returns to normal.
               Taper prednisone by 5-10 mg on a monthly basis until the lowest dose that controls
                 the disease is reached.
       o Immunosuppressive agents are indicated in patients who do not improve with steroids
          within a reasonable period (ie, 4 wk) or in whom adverse effects from corticosteroids
          develop.
               Patients with poor prognostic indicators, such as dysphagia or dysphonia, are likely
                 to require immunosuppressive agents.
       o Methotrexate is the second-line agent however, azathioprine, cyclophosphamide,
          chlorambucil, and cyclosporine have been used with varying success as second-line agents
          for polymyositis.
       o Obtain baseline bone marrow, liver function and pulmonary function tests before initiating
          immunosuppressive therapy.
       o Intravenous immunoglobulin (IVIG) has been used for the short-term treatment of steroid-
          resistant cases of polymyositis.
       o The use of TNF inhibitors in refractory cases has demonstrated some success in recent
          studeis but not yet standard therapy.
       o Dermatomyositis treated with rituximab (anti-CD20 monoclonal antibody) yield of positive
          results but has not been duplicated in polymyositis patients.
 Dermatomyositis
       o Intravenous immunoglobulin (IVIG) has been used for the short-term treatment of steroid-
          resistant cases of polymyositis.
       o The use of TNF inhibitors in refractory cases has demonstrated some success in recent
          studeis but not yet standard therapy.
       o Dermatomyositis treated with rituximab (anti-CD20 monoclonal antibody) yield of positive
          results but has not been duplicated in polymyositis patients.
   Diagnostic Criteria
        Progressive proximal symmetrical weakness
        Elevated levels of muscle enzymes
        Abnormal findings on electromyography
        Abnormal findings on muscle biopsy.
        Cutaneous disease, which may or not preclued the muscle changes
   Sub-Sets of DMS
       o Amyopathic dermatomyositis [ADM], or dermatomyositis sine myositis--- dermatomyositis
          that affects only the skin and patients have normal muscle enzyme levels without therapy
       o Postmyopathic dermatomyositis: a subset of patients with dermatomyositis who have
          controlled myopathy but severe and sometimes debilitating skin disease.
   Therapy
        o Therapy for the muscle destruction associated with inflammation of dermatomyositis
           involves the use of corticosteroids, with or without an immunosuppressive agent.
           Prednisone and methotrexate
        o Rituximab may be useful in the treatment of muscle disease of dermatomyositis and has
           had mixed results in treatment of skin disease.
        o The skin disease is treated with sun avoidance, sunscreens, topical corticosteroids,
           antimalarial agents, methotrexate, mycophenolate mofetil, or intravenous (IV)
           immunoglobulin.
        o The prognosis depends on the severity of the myopathy, the presence of malignancy,
           and/or the presence of esophageal and/or cardiopulmonary involvement.
        o Residual weakness is common, even in patients who fully recover.
   Pathophys
        o Mediated by a humoral attack against the muscle capillaries and small arterioles and an
           ongoing microangiopathy.
        o Complement is deposited at vessel lining, preparing the cell for destruction in the antibody-
           mediated inflammatory disease generated by products of by B cells and CD4 helper cells.
        o The capillaries are destroyed, and the muscles undergo microinfarction.
        o Perifascicular atrophy occurs followed by necrosis along with degenerative changes
           throughout the muscle fibers.
   Etiology
        o Genetics may predispose to but rarely occurs in multiple family members.
        o Human leukocyte antigen (HLA) types (DR3, DR5, DR7) are associated in patients with the
           condition.
        o Polymorphisms of tumor necrosis factor may be involved; specifically, the presence of the -
           308A allele is linked to photosensitivity in adults and calcinosis in children.
        o Immunologic abnormalities are common and patients frequently have circulating
           autoantibodies and abnormal T-cell activity.
        o Antinuclear antibodies (ANAs) and antibodies to cytoplasmic antigens (ie, antitransfer RNA
           synthetases) may be present, their role in pathogenesis is uncertain.
        o Infectious agents, including viruses (eg, coxsackievirus, parvovirus, echovirus, human T-
           cell lymphotropic virus type 1 [HTLV-1], HIV) and Toxoplasma and Borrelia species, have
           been suggested as possible triggers.
        o Drug-induced dermatomyositis has been reported with hydroxyurea , penicillamine, statin
           drugs, quinidine, and phenylbutazone
   Epi
        o The estimated incidence of dermatomyositis is 9.63 cases per million population.
        o Dermatomyositis can occur in patients of any age.
            Two peak ages of onset exist: in adults, the peak age of onset is approximately 50
               years, whereas in children, the peak age is approximately 5-10 years.
        o Dermatomyositis and polymyositis are twice as common in women as in men.
   Prognosis
        o Most patients with dermatomyositis develop residual weakness and disability.
        o Children with severe dermatomyositis may develop contractures.
        o The disease may spontaneously remit in as many as 20% of affected patients.
        o About 5% of patients have a fulminant progressive course with eventual death.
        o Patients with dermatomyositis who have malignancy, cardiac involvement, or pulmonary
           involvement or who are elderly (ie, > 60 years) have a poorer prognosis.
        o Calcinosis is very rare in adults but is more common in children.
   Hx/PE
         o In up to 40% of individuals with dermatomyositis, skin disease is the sole manifestation at
           onset.
         o Muscle disease may occur concurrently, may precede the skin disease, or may follow the
           skin disease by weeks to years.
         o Muscle involvement manifests as proximal muscle weakness.
         o Noted by muscle fatigue or weakness when climbing stairs, walking, rising from a sitting
           position, combing their hair, or reaching for items in cabinets that are above their
           shoulders.
         o Muscle tenderness may occur but is not a regular feature of dermatomyositis.
         o Systemic manifestations such as arthralgia, arthritis, dyspnea, dysphagia, arrhythmia, and
           dysphonia often occur.
         o Malignancy is possible in any patient with dermatomyositis, but it is much more common
           in adults older than 60 years.
         o Children tend to have extramuscular manifestations, especially gastrointestinal (GI) ulcers
           and infections, more frequently than adults do.
   PE
         o The characteristic and possibly pathognomonic cutaneous features of dermatomyositis are
           a heliotrope (ie, blue-purple) discoloration on the upper lids and a raised, violaceous, scaly
           eruption on the knuckles (ie, Gottron papules).
         o Commonly patients exhibit a flat, red rash involving the face and upper trunk.
         o The erythematous lesions may result in scaling, pigmentation, and depigmentation of the
           skin, producing a shiny appearance.
         o The rash may involve other body surfaces, including knees, elbows, neck, anterior chest (ie,
           V sign), or back and shoulders (ie, shawl sign)
         o Dilated capillary loops at the base of the fingernail are characteristic of dermatomyositis.
         o The cuticles may be irregular and thickened, and the palmar and lateral surfaces of the
           fingers may become rough and cracked.
         o Sun exposure can exacerbate the rash.





   DMS
      o Muscle disease commonly manifests as a proximal symmetrical muscle weakness.
      o Muscle pain and tenderness are variable but observed early in the course of the disease.
      o Sensation and neuro function is normal with deep tendon reflexes preserved until the
        muscle is severely atrophic.
      o Joint swelling occurs in some patients with dermatomyositis.
         The small joints of the hands are the most frequently involved.
         The arthritis associated with dermatomyositis is nondeforming.
   DDx
       o Discoid Lupus Erythematosus
       o Lichen Myxedematosus
       o Lichen Planus
       o Parapsoriasis en plaques
       o Psoriasis vulgaris
       o Rosacea
       o Sarcoidosis
       o Systemic Lupus Erythematosus (SLE)
   Lab Studies
       o The most sensitive/specific enzyme is elevated creatine kinase (CK), but aldolase studies,
           aspartate aminotransferase [AST] or lactic dehydrogenase [LDH]) may also yield abnormal
           results.
       o A positive antinuclear antibody (ANA) finding is common but none specific in patients with
           dermatomyositis.
       o Anti–Mi-2 antibodies are highly specific for dermatomyositis, but sensitivity is low at only
           25%.
            These autoantibodies are associated with acute-onset classic dermatomyositis with the
               V-shaped and shawl rash (poikiloderma) and a relatively good prognosis.
       o Anti–Jo-1 (antihistidyl transfer RNA [t-RNA] synthetase) antibodies are far more relilable
           in patients with polymyositis than in patients with dermatomyositis.
            They are associated with pulmonary involvement (interstitial lung disease), Raynaud
               phenomenon, arthritis, and mechanic’s hands.
   Muscle biopsy
       o Useful in diagnosis and in differentiating steroid myopathy from active inflammatory
           myopathy when patients have been on corticosteroid therapy but are still weak
       o Muscle biopsy in patients with dermatomyositis reveals perivascular and interfascicular
           inflammatory infiltrates with adjoining groups of muscle fiber degeneration/regeneration.
            This contrasts with polymyositis infiltrates, which are mainly intrafascicular
               (endomysial inflammation) with scattered individual muscle fiber necrosis.
   Tx
       o The muscle component of the inflammatory disease is treated by administering
           corticosteroids, with or without an immunosuppressive agent.
       o The skin disease is treated by avoiding sun exposure and by using sunscreens, systemic or
           topical corticosteroids, antimalarial agents, or an agent such as methotrexate or
           mycophenolate mofetil.
       o The use of drugs such as methotrexate, azathioprine, cyclophosphamide, cyclosporine,
           mycophenolate mofetil, leflunomide, and chlorambucil has been reported to be steroid-
           sparing.
       o The use of monthly high-dose intravenous immune globulin (IVIG) for 6 months has proved
           beneficial in the short term for both muscle and skin disease in addition to other systemic
           manifestations.
       o Rituximab, a chimeric antibody directed against CD20+ B cells, may be effective in treating
           muscle necrosis and atropy.
 Polymyalgia Rheumatica (PMR)
       o CLINICAL DIAGNOSIS********************
       o Polymyalgia rheumatica is a clinical diagnosis characterized by proximal myalgia of the hip
         and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour.
       o Approximately 15% of patients with polymyalgia rheumatica develop giant cell
         arteritis (GCA), and approximately 50% of patients with giant cell arteritis have
         associated polymyalgia rheumatica.
        o Usually self-limited with prompt diagnosis and adequate therapy.
        o The average length of disease is 3 years.
        o Exacerbations may occur if steroids are tapered too rapidly, and relapse is common,
           affecting up to 25% of all treated patients.
   Pathophys
        o HLA-DR4 is found with increased frequency in patients with polymyalgia rheumatica and
           in those with giant cell arteritis.
        o Systemic monocyte activation is characteristic of both conditions.
        o Both diseases show a sequence polymorphism encoded within the second hypervariable
           region of the HLA-DRB1 gene.
        o Patients with polymyalgia rheumatica often have elevated levels of interleukin-2 (IL-2) and
           interleukin-6 (IL-6), especially during flares.
        o Environmental factor s trigger an auto-immune process resulting in monocyte activation
           and the production of cytokines that induce polymyalgia rheumatica and giant cell arteritis.
        o Although polymyalgia rheumatica causes severe pain in the proximal muscle groups, no
           evidence of pathology or necrosis is present on muscle biopsy. Shoulder >hip
        o Muscle strength and electromyographic findings are normal.
   Etiology and Epi
        o More common among northern Europeans, which indicates a genetic predisposition.
        o Other risk factors are age of 50 years or older and the presence of giant cell arteritis.
        o Many investigators believe that nonerosive synovitis and tenosynovitis are responsible for
           many symptoms of polymyalgia rheumatica.
        o Whites are affected more than other ethnic groups.
        o Polymyalgia rheumatica is twice as common in females.
        o Polymyalgia rheumatica rarely affects persons younger than 50 years.
        o The median age at diagnosis is 72 years.
   Hx
        o Disease onset is abrupt in about 50% of patients.
        o In most patients, symptoms appear first in the shoulder girdle. In the remainder, the hip or
           neck is involved at onset.
        o Symptoms may be unilateral initially but they usually become bilateral within a few
           weeks.
        o Criteria for diagnosis are as follows:
            Age 50 years or older at onset
            Bilateral aching and morning stiffness for at least 1 month and involving at least 2 of 3
               areas: neck or torso, shoulders or arms, hips or thighs
            Westergren erythrocyte sedimentation rate (ESR) 40 mm/h or greater
            Prompt response of symptoms to corticosteroids (15 mg/d)
        o Systemic findings are as follows:
                Low-grade fever and weight loss
                Malaise, fatigue, and depression
                Difficulty rising from bed in the morning
                Difficulty getting up from the toilet
                Difficulty completing daily life activities
                High, spiking fevers (rare)
        o Musculoskeletal findings are as follows:
                Morning stiffness for more than 1 hour, often more prolonged
                Muscle stiffness after prolonged inactivity
                Carpal tunnel syndrome (in about 15% of patients)
                Distal extremity swelling (uncommon)
                 Possible development of arthralgia and myalgia up to 6 months after onset of
                  systemic symptoms
   PE
       o Normal muscle strength; no muscle atrophy
       o Pain in the shoulder and hip with movement without significant clinical swelling; active
         range of motion may be decreased because of pain
       o Transient synovitis of the knee, wrist, and sternoclavicular joints
       o Tenderness to palpation with decreased active range of motion in the musculature of the
         proximal hip/leg and/or shoulder/arm girdle
       o In later stages, disuse muscle atrophy with proximal muscle weakness may occur.
         Contractures of the shoulder capsule may lead to limitation of passive and active
         movement.
   DDx
       o Bursitis/tendinitis
       o Cervical spondylosis
       o Dermatomyositis
       o Fibromyalgia
       o Myopathy
       o Osteoarthritis
       o Parkinson disease
       o Shoulder disorders (eg, shoulder synovitis, rotator cuff tendinitis, and subdeltoid bursitis)
       o Amyloidosis, AA (Inflammatory)
       o Depression
       o Giant Cell Arteritis
       o Multiple Myeloma
       o Polymyositis
       o Rheumatoid Arthritis
   Eval
       o Serum interleukin-6 (IL-6) [occassionall IL-2] levels are elevated and often closely parallel
         inflammatory activity of the disease.
       o In the patient who has synovitis with effusions, synovial fluid analysis reveals signs of mild
         inflammation, including poor mucin clotting.
       o MRI of the shoulder reveals subacromial and subdeltoid bursitis and glenohumeral joint
         synovitis in the vast majority of patients.
       o MRI of the hands and feet demonstrates inflammation of the tendon sheaths in many
         patients.
       o Symptomatic vasculitis in cranial and extracranial vessels is rare in polymyalgia
         rheumatica although sub clinical findings can be shown with PET scan in up to 1/3 of
         patients.
       o The creatine kinase level is normal which differentiates polymyalgia rheumatica from
         polymyositis and other primary myopathic disorders.
       o The erythrocyte sedimentation rate (ESR) is the most sensitive diagnostic study for
         polymyalgia rheumatica, although it is not specific.
          The ESR is frequently elevated and greater than 40 mm/h, but it can exceed 100
             mm/hr.
       o The C-reactive protein level is often elevated and may parallel the ESR.
          This is a more sensitive test than ESR for the diagnosis of PMR.
   Temporal Arteritis
       o Temporal artery biopsy has a very low yield with isolated polymyalgia rheumatica.
         o TAB is not indicated in patients with mild symptoms of polymyalgia rheumatica that are of
           recent onset or in patients who have remained stable over a long period (1 y or longer
           without current or previous clinical evidence of arteritis).
         o Patients should be monitored for symptoms or signs of temporal arteritis after treatment
           initiation because low-dose corticosteroids do not prevent progression of polymyalgia
           rheumatica to giant cell arteritis.
   Tx
       o Polymyalgia rheumatica is a chronic, self-limited disorder. Therapeutic goals are to control
          painful myalgia, to improve muscle stiffness, and to resolve constitutional features of the
          disease.
       o Corticosteroids are considered the treatment of choice because they often cause complete
          or near-complete symptom resolution and reduction of the erythrocyte sedimentation rate
          (ESR) to normal.
           No definite evidence demonstrates that corticosteroids (or any other therapy) alter the
              natural history of polymyalgia rheumatica.
           The low-dose corticosteroids used in polymyalgia rheumatica are almost certainly
              ineffective in the prevention of vasculitis progression.
       o Remission of polymyalgia rheumatica achieved with a 15 mg/d dose of prednisone
          achieved in most patients.
           A slow tapering of the prednisone, less than 1 mg/month, was associated with fewer
              relapses
       o 50-75% of patients can discontinue corticosteroid therapy after 2 years of treatment.
       o Nonsteroidal anti-inflammatory drugs (NSAIDs) may provide supplemental pain relief.
           NSAIDs may be helpful in later stages of corticosteroid dosage tapering.
           NSAIDs generally have no effect on ESR.
   Pharm Agents
       o Prednisone: an anti-inflammatory and immunosuppressive agent used in the treatment of
          autoimmune disorders.
       o Prednisone stabilizes the lysosomal membrane and suppresses lymphocytes, reducing
          cytokine and antibody production
       o Inhibition of the function of leukocytes and tissue macrophages, which diminishes their
          ability to respond to antigens and mitogens
       o Inhibition of phospholipase A2, resulting in decreased prostaglandin and leukotriene
          synthesis
       o Inhibition of cyclooxygenase II expression, which may be the enzyme more involved in the
          inflammatory effects of eicosanoids
       o Decreased activity of kinins and decreased histamine release by basophils, leading to
          decreased capillary permeability.
   Tx Agents
       o Methotrexate : an antimetabolite that inhibits DNA synthesis and cell reproduction.
           Methotrexate has benefits in both muscle and skin disease. Methotrexate ameliorates
              symptoms of inflammation (eg, pain, swelling, stiffness). A satisfactory response is
              typically seen 3-6 weeks after administration.
       o Azathioprine: a purine analogue that inhibits purine synthesis, resulting in inhibition of
          DNA, RNA, and protein synthesis.
           It decrease proliferation of immune cells, thereby leading to lower autoimmune activity.
              It has few, if any, effects on the skin.
       o Mycophenolate: It inhibits purine synthesis and proliferation of human lymphocytes.
           Useful for both skin and muscle disease
       o Chlorambucil: alkylates and cross-links strands of DNA, inhibiting DNA replication and
           RNA transcription.
       o Cyclosporine: a cyclic polypeptide that suppresses cell-mediated immune reactions and, to
           a lesser extent, humoral immunity, allograft rejection, experimental allergic
           encephalomyelitis, and graft versus host disease.
            Selectively inhibits the transcription of interleukin 2, predominantly in helper
               lymphocytes.
       o Cyclophosphamide: an alkylating agent, interferes with the cross-linking of DNA, which
           interfers with the growth of normal cells such as lymphocytes and neoplastic cells.
       o Hydroxychloroquine: Hydroxychloroquine inhibits chemotaxis of eosinophils and
           locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
            Morbilliform drug reactions are more common in patients with dermatomyositis than
               in those with other collagen vascular diseases.
   Biological Tx Agents
       o Etanercept: binds specifically to TNF and blocks its interaction with cell-surface TNF
           receptors, rendering TNF biologically inactive.
       o Infliximab: binds to soluble and transmembranous forms of TNF-alpha, rendering TNF
           biologically inactive.
       o Rituximab: a genetically engineered chimeric murine/human monoclonal antibody
           directed against the CD20 antigen found on the surface of normal and malignant B
           lymphocytes.
       o Immune globulin, intravenous: IVIG is useful for patients in whom corticosteroids and
           immunosuppressants have failed.

				
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