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 : 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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