CM-MSK Exam 2 Notes
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
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)
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)
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
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
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
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
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
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
b. Glenoid labrum: cartilage rim around edge. Makes shoulder socket a little deeper, more
i. Superior portion: relatively mobile
ii. Inferior: immobile
c. Capsule/Ligaments: “shrink wrap” that holds humeral head in place like wheel/axel.
1. Superior glenohumeral
2. Middle glenohumeral
3. INFERIOR GLENOHUMERAL (most important ligamentous stabilizer when
shoulder is abducted and externally rotated)
d. Joint Cohesion: limited volume of fluid, airtight
e. Intraarticular pressure: negative pressure holds joint together, hole in capsule leads to
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
i. First 30-45degrees of Abduction has little movement of scapula, after that RATIO IS
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)
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
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
a. Check sensation, pulses.
b. Shoulder Dislocation
i. Anatomical considerations
ii. Etiology and types
iv. Relocation techniques
v. Complications/assoc. pathology
10. Shoulder Instability (differentiate instability based on mnemonics)
ii. Unidirectional dislocation with an association
iii. Bankart lesion, likely to respond to
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,
b. Posterior: Arm forward flexed, internally rotated, adducted with posterior directed force;
secondary electric shock/seizure; direct (car) or repetitive trauma (football) to anterior
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
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
i. Arm elevated and hyperabducted (resting on back of head)
13. General Laxity
14. Instability Testing
a. Anterior Apprehension Test (variation of “relocation test”)
15. Axillary nerve evaluation Test
a. Intact sensation over lateral deltoid, full arm extension strength (isolates posterior deltoid
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
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
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
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
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
f. Middle 1/3 fractures
g. New emphasis on restoring anatomic position and length
Non-Surgical Injuries of Hand and Wrist
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
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,
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
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
a. Reduce when appropriate (fingers more appropriate to reduce in field than wrist)
b. Pre- and post- reduction films helpful
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
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
a. Displaced, transverse, two-bone forearm shaft fractures
b. Comminuted, angulated, displaced fx of the distal radius
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
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
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
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
b. Dis: hardware complications, adhesions and finger stiffness, second procedure to remove
c. Aftercare: begin PT immediately, remove plate when fx has healed
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
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
Injuries to Elbow and Forearm
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
a. Bicep tendonitis, triceps tendinits, olecranon bursitis, nursemaids, MCL sprain, medial
3. Lateral Epicondylitis: aka Tennis elbow
a. Faulty mechanics, poorly fitted equipment, repetitive job at work
b. Difficulty with wrist extension
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
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,
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)
e. Tx: rest, NSAID, rehab
8. Median Nerve Injury: typically by blunt trauma, penetrating wounds
a. Innervates the thenar compartment, allowing for fine control of the pincer grip (APE
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
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
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
b. PRICE: protection, RICE
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
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
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
e. Closed manipulation and casting
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!
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
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
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
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
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
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
10. Referred pain
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
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
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
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
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
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
d. Strength: 5/5 Quads, no atrophy
e. Common Problems: Labral Tear
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
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
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
1. X-rays (healing); usually (-) for diagnosis
2. Bone-scan (+) 72 hours after injury
1. Similar sensitivity
2. Better specificity
1. Strict rest (?!)
a. Tension vs compression side
c. Avulsion Fx
1. ASIS (sartorius),
2. AIIS (rectus),
3. Ischial (hamstring)
1. Chronic, Acute concentric/eccentric
1. Males, adolescents, local pain, limit motion
iv. Physical Findings: tenderness, limit ROM & weakness on MMT
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
iii. History: Severe groin pain, diff standing, cutting
iv. Physical Findings: Tender pubic symphysis, weak adductors, pain with one-legged
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
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
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-
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
vi. Imaging: x-ray only to rule out other injury
vii. Treatment: Rest, Ice, NSAIDS, Injection, Correct biomechanics
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
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
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
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
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
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
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
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
** 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!
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)
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 Base of thumb: OA
o Reduction of joint loading, orthotic
o Exercise, PT
o Drug Therapy
o Intra-articular therapy (corticosteroids, hyaluronic)
o EXERCISE (strength, aerobic, ROM, agility, neck and back strength)
Homeopathic: Tumeric, MSM, Boswellia, Cetyl Myristoleate
Gemmotherapy: Pinus monatana, Ribes nigrum, Vitis vinifera
Intra-articular: corticosteroids (long-term: joint destructive), hyaluronic acid
Glucosamine/chondroitin has been proven to work for some; fairly safe. Watch out
if you are diabetic, otherwise well-tolerated
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
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
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
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])
Bone and Joint Infections
Osteomyelitis: Destruction of bone (infection)
o Acute (~10 days)
Penetration, hematogenous spread, contiguous spread
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
Bone resistant to infection
S. aureus: skin colonizer, cause cellulitis and bacteremia, receptors for fibronetin
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)
Adjacent soft tissue infection
Sickle cell disease (devitalized bone)
Congenital defects in phagocyte function
o Osteomyelitis in Diabetes: Neuropathy leads to undiscovered foot ulceration
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
Probe to bone (diabetic ulcers easier…)
Blood work (CBC, ESR elevated, Blood culture (50% + in hematogenous)
Aspirate cultures – normal in 25% cases
o What to do?
Treat symptoms (oral antibiotics, then IV)
Work-ups, imaging, etc.
Cellulitis, gout/pseudogout, neuropathic joint, fx, aseptic bone infaction, sickle cell
o Radiographic Diagnosis
Early xray normal (first signs, soft tissue swelling)
Early positive due to osteoclastic activity at 14-21 days (usually periosteal
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
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
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
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
Apetite related arthropathy
Dx early is difficult, erythema, warm, swollen, loss of ROM
Direct penetration (trauma, surgery, bites, injection)
Extension into joint from adjacent infection
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)
Fluid for culture (WBC>50,000, Gram stain, cultures)
Don’t go through skin infection to get to joint!!
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
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
In patients with RA, there is documented abnormal production of numerous
cytokines, chemokines, and other inflammatory mediators that add to destructive
(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])
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)
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
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
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!)
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
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
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
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
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
Small joints of hands/feet in symmetric distribution, inflamm, swelling, tender,
warm, decreased ROM
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
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
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
Midfoot disease leads to loss of normal arch contour with flattening of the
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
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
Myocarditis, coronary vasculitis, valvular disease, and conduction defects are
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
The liver is often affected in patients with Felty syndrome (ie, RA, splenomegaly, and
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
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
o Class III - Able to perform usual self-care activities but limited in vocational and avocational
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 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 Lyme Disease
o Myelodysplastic Syndrome
o Degenerative Joint Disease
o Paraneoplastic Syndromes
o Gout and Pseudogout
o Polymyalgia Rheumatica
o Psoriatic Arthritis
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
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
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.
o Xray first, MRI more sensitive (cervical subluxation, radiculopathy), Bone scan only for
determining degree of inflammation
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
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
Liver and bone marrow toxicity (MTX, SSZ, leflunomide, azathioprine, gold
Renal toxicity (cyclosporin A, parenteral gold salts, D-penicillamine), pneumonitis
Allergic skin reactions (gold compounds, SSZ), autoimmunity (D-penicillamine, SSZ,
Infections (azathioprine, cyclosporin A)
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
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 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
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.
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
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
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
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
Polyarticular (over 5 joints)
Pauciarticular (1-4 joints)
Systemic (constitutional symptoms and joint involvement)
o Other types (juvenile akylosing spondy, psoriatic arth) are spondyloarthropathies
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).
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
o The EULAR criteria for JCA recognize the following subtypes, based on characteristics at
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
o The IL2RA/CD25 gene has been implicated as a JIA susceptibility locus, as has the VTCN1
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
o Macrophages and T-cell invasion are associated with the release of cytokines, which evoke
o Approximately 300,000 children in the United States are estimated to have some type of
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
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
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.
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.
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
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.
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
o Arthritis of the temporal-mandibular joint (TMJ) may lead to micrognathia
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
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
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
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
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-
o Patients who show inadequate response to TNF-alpha inhibitor treatment can be started
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
o MAS often responds to cyclosporin A, and some case reports have detailed a response to
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
o Infliximab can be effective in some patients who are resistant to immunosuppressive
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
o The pediatric dosage is not established for patients younger than 6 years.
o Adalimumab is a recombinant human IgG1 monoclonal antibody that is specific for human
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
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.
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.
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
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
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.
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
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).
o Increased myositis is associated with human leukocyte antigen (HLA) haplotypes A1, B8,
o Environmental triggers include the following:
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
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
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.
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
o Poor prognostic factors include the following:
African American race
Interstitial lung disease
Presence of anti-Jo-1 (lung disease) and anti-SRP antibodies (severe muscle disease,
Cardiac and pulmonary involvement
o Complications of polymyositis may include the following:
Interstitial lung disease, pneumoniae,
Cardiac muscle disease
Dysphagia and reflux
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
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
Polymyositis has been associated with other connective-tissue diseases, including
Systemic lupus erythematosus
Mixed connective-tissue disease
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
Sensory examination findings are normal.
The tendon reflexes are preserved, but they may be absent in severely weakened or
Primary cardiac abnormalities due to myocarditis may be present in a few patients.
Conditions to consider in the differential diagnosis of polymyositis include the
Hypokalemia (and muscle weakness)
Overlap connective-tissue diseases
Drug-induced myopathy may result from the following:
Ketoconazole and other azole antifungal agents
Statin/3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase
inhibitors (CK gets very high)
Amyotrophic Lateral Sclerosis (ALS) in Physical Medicine and Rehabilitation
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
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
o Signal-recognition particle (SRP) antibodies - Approximately 4% of patients with
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
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
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
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
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
o Obtain baseline bone marrow, liver function and pulmonary function tests before initiating
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.
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.
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.
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)
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.
o Mediated by a humoral attack against the muscle capillaries and small arterioles and an
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.
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
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
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.
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.
o In up to 40% of individuals with dermatomyositis, skin disease is the sole manifestation at
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
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.
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.
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.
o Discoid Lupus Erythematosus
o Lichen Myxedematosus
o Lichen Planus
o Parapsoriasis en plaques
o Psoriasis vulgaris
o Systemic Lupus Erythematosus (SLE)
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
o A positive antinuclear antibody (ANA) finding is common but none specific in patients with
o Anti–Mi-2 antibodies are highly specific for dermatomyositis, but sensitivity is low at only
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.
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.
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
o The use of drugs such as methotrexate, azathioprine, cyclophosphamide, cyclosporine,
mycophenolate mofetil, leflunomide, and chlorambucil has been reported to be steroid-
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
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.
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.
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
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
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
o Cervical spondylosis
o Parkinson disease
o Shoulder disorders (eg, shoulder synovitis, rotator cuff tendinitis, and subdeltoid bursitis)
o Amyloidosis, AA (Inflammatory)
o Giant Cell Arteritis
o Multiple Myeloma
o Rheumatoid Arthritis
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
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
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
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.
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.
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
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
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.
o Prednisone: an anti-inflammatory and immunosuppressive agent used in the treatment of
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
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.
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
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
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
o Rituximab: a genetically engineered chimeric murine/human monoclonal antibody
directed against the CD20 antigen found on the surface of normal and malignant B
o Immune globulin, intravenous: IVIG is useful for patients in whom corticosteroids and
immunosuppressants have failed.