290 Vasculitis SPECIFIC ENTITIES (CONT’D) mg/month) every weeks until tapered off Use of prednisone greater than 15 mg decreases the diagnostic specificity Relapse is frequent GIANT CELL ARTERITIS/TEMPORAL ARTERITIS ASSOCIATIONS older age, polymyalgia rheumatica in 30 50% CLINICAL FEATURES systemic vasculitis of the large and medium arteries This causes headache, amaurosis fugax, diplopia, jaw claudication, painful scalp nodules, and tender temporal artery Extra cranial GCA involves aorta in 10 15% of cases ACR DIAGNOSTIC CRITERIA age >50, new onset headache, abnormal temporal artery, ESR SPECIFIC ENTITIES (CONT’D) >50 mm/h, abnormal temporal artery biopsy Need three of five criteria (sens 94%, spc 91%) TREATMENTS if no ocular symptoms, prednisone 40 60 mg PO daily Â1 month, taper to 7.5 15 mg daily over months, may continue for several years (monitor symptoms, signs, and CRP) If ocular symptoms present, start methylpred nisolone g IV daily Â3 days, then prednisone 80 mg PO daily and taper over time Initiate ther apy before biopsy if high index of suspicion Con sider methotrexate if steroid sparing therapy required ASA 81 mg PO daily is recommended to reduce vascular complications RATIONAL CLINICAL EXAMINATION SERIES: DOES THIS PATIENT HAVE TEMPORAL ARTERITIS? LR+ LR History Jaw claudication 4.2 0.72 Diplopia 3.4 0.95 Temporal headache 1.5 0.82 Any headache 1.2 0.7 Unilateral visual loss 0.85 1.2 Any visual symptom 1.1 0.97 Vertigo 0.71 1.1 Anorexia 1.2 0.87 Weight loss 1.3 0.89 Arthralgia 1.1 Fatigue 1.2 0.94 Fever 1.2 0.92 Myalgia 0.93 1.1 Polymyalgia rheumatica 0.97 0.99 Physical Beaded temporal artery 4.6 0.93 Prominent temporal artery 4.3 0.67 Tender temporal artery 2.6 0.82 Absent temporal artery pulse 2.7 0.71 Any temporal artery 0.53 Scalp tenderness 1.6 0.93 Optic atrophy or ischemic optic neuropathy 1.6 0.8 Any fundoscopic abnormality 1.1 1.0 Synovitis 0.41 1.1 Male gender 0.83 White race 1.1 Laboratory investigations Anemia 1.5 0.79 ESR abnormal 1.1 0.2 ESR >50 mm/h 1.2 0.35 ESR >100 mm/h 1.9 0.8 APPROACH ‘‘when taking a history in a patient with possible temporal arteritis, jaw claudication and diplopia substantially increase the probability of positive biopsy results No historical findings help rule out the diagnosis by their absence Among physical examination findings, synovitis makes the diagnosis of temporal arteritis less likely, while beaded, prominent, enlarged, and tender temporal arteries each increase the likelihood of positive biopsy results While these findings increase the chance of having temporal arteritis, they are variably sensitive from 16% (beaded temporal artery) to 65% (any temporal artery abnormality) The results of tests of ESR alter the likelihood of positive biopsy results A normal ESR or ESR 100 mm/hr have a modestly increased likelihood of biopsy proven temporal arteritis The prevalence of temporal arteritis in the general population is 4 kg since illness, livedo reticularis, testicular pain or tenderness, myalgias, weakness or leg tender ness, mononeuropathy or polyneuropathy, dia stolic blood pressure >90 mmHg, elevated urea >14 mmol/L [>39 mg/dL] or Cr >132 mmol/L [>1.45 mg/dL], HBsAg or HBsAb positive, arter iographic abnormality (aneurysms or occlusions of the visceral arteries, not due to arteriosclero sis, fibromuscular dysplasia, or other non inflam matory causes), biopsy of small or medium sized artery containing PMN Need of 10 criteria (sens 82%, spc 87%) TREATMENTS steroids, cyclophosphamide MICROSCOPIC POLYANGIITIS (MPA) PATHOPHYSIOLOGY necrotizing vasculitis of the small vessels Frequent glomerulonephritis and lung involvement CLINICAL FEATURES renal (RPGN), pulmonary (hemoptysis, hemorrhage) GI, skin, and neurologic symptoms as in PAN p ANCA positive TREATMENTS steroids, cyclophosphamide WEGENER’S GRANULOMATOSIS PATHOPHYSIOLOGY systemic vasculitis of the med ium and small arteries, venules, and arterioles Also necrotizing granulomas involving upper and lower respiratory tracts and kidneys Associated with sinusitis and c ANCA (autoantibodies against pro teinase 3) CLINICAL FEATURES wELKSw Ears and nose, Lungs, Kidneys, and Skin involvement ACR DIAGNOSTIC CRITERIA nasal or oral inflamma tion/ulcers, abnormal CXR (nodules, fixed infil trates, cavities), microhematuria (>5 RBC/HPF) or red cell casts in urine sediment, granulomatous inflammation on biopsy Need two of four criteria for diagnosis (sens 88%, spc 92%) TREATMENTS steroids, cyclophosphamide, metho trexate, rituximab SPECIFIC ENTITIES (CONT’D) CHURG STRAUSS SYNDROME PATHOPHYSIOLOGY systemic vasculitis of the med ium and small arteries, typically involving the lung and skin Also vascular and extravascular granulo matosis with necrosis Associated with asthma and p ANCA (autoantibodies against myeloperoxi dase), eosinophilia, and " IgE and ESR ASSOCIATIONS leukotriene type I receptor antagonists CLINICAL FEATURES pneumonic infiltrate, skin rash, myocarditis, peripheral neuropathy, and nephropathy ACR DIAGNOSTIC CRITERIA asthma, eosinophilia >10%, mono or polyneuropathy, pulmonary infil trates (non fixed), paranasal sinus abnormality, extravascular eosinophils Need four of six criteria for diagnosis (sens 85%, spc 99.7%) TREATMENTS steroids, cyclophosphamide HENOCH SCHONLEIN PURPURA PATHOPHYSIOLOGY systemic vasculitis of small vessels characterized by IgA containing immune complex deposition in tissues ACR DIAGNOSTIC CRITERIA palpable purpura, age 50 U/mL and fulfilling other criteria, rheumatoid arthritis is diag nosed without need for anti CCP However, if rheu matoid factor 95%) and chronic active hepatitis Usually not elevated in drug induced lupus UTILITY associated with lupus nephritis and disease activity in SLE (most useful for following disease) ANTI SMITH (very specific) DISORDERS SLE Usually not elevated in drug induced lupus UTILITY SLE (sens 30%, spc >95%) Associated with lupus nephritis ANTI RNP DISORDERS SLE, mixed connective tissue disease UTILITY associated with milder SLE ANTI HISTONE DISORDERS drug induced lupus (sens >90%, very spc), SLE (sens >50%) C3, C4 DISORDERS decreased in SLE, cryoglobulinemic vasculitis, Henoch Schonlein purpura UTILITY associated with lupus nephritis and dis ease activity in SLE and cryoglobulinemic vasculitis RIM HOMOGENOUS SCLERODERMA ANTI SCL 70 (TOPOISOMERASE I) (very specific) DISORDERS scleroderma (sens 20 30%, very spc) UTILITY associated with disease activity ANTICENTROMERE DISORDERS CREST (sens 90%), idiopathic Raynaud’s (sens 25%) SJOGREN’S SYNDROME LUPUS ANTINUCLEAR ANTIBODIES (ANA) (non specific but most sensitive test for SLE) DISORDERS SLE (sens >99%), mixed connective tissue disease (sens >95%), Sjogren’s syndrome (sens 75%), inflammatory myopathies (sens >75%), scleroderma (sens >60 90%), rheumatoid arthritis (sens 15 35%), and normal elderly ANTI RO (SS A) DISORDERS Sjogren’s syndrome (sens 75%), SLE (sens 25%) UTILITY associated with sicca in other connective tissue disorders, extraglandular disease in Sjog ren’s syndrome, heart block in neonates with anti Ro positive mothers, cutaneous lupus rash, photosensitivity, and thrombocytopenia in SLE 293 Approach to Serologies SJOGREN’S SYNDROME (CONT’D) INFLAMMATORY MYOPATHIES (CONT’D) ANTI LA (SS B) DISORDERS Sjogren’s syndrome (sens 40%), SLE (sens 10%) UTILITY associated with anti Ro and benign course in SLE if no other autoantibody present except ANA INFLAMMATORY MYOPATHIES ANTI JO synthetase antibodies against t RNA histidyl polymyositis (sens 30%) associated with deforming arthritis, ‘mechanic’s hands’’, Raynaud’s, and pulmonary fibrosis in dermatomyositis and polymyositis ANTI MI DISORDERS dermatomyositis (sens 5%) UTILITY associated with V sign, shawl sign, cuti cular overgrowth, good response to therapy, and good prognosis DISORDERS UTILITY ANTI SRP antibodies against antisignal recogni tion protein DISORDERS dermatomyositis and polymyositis VASCULITIS C ANCA autoantibodies against proteinase Con firm by testing for antiproteinase DISORDERS Wegener’s granulomatosis (sens >80%) is the most common disorder P ANCA autoantibodies against myeloperoxidase (non specific) May need to confirm with testing for anti myeloperoxidase (MPO) DISORDERS Churg Strauss (sens 65%), idiopathic crescentic glomerulonephritis (sens 65%), micro scopic polyangiitis (sens 45%), polyarteritis nodosa (sens 15%), Wegener’s granulomatosis (sens 10%) Boutonniere, Swan neck, subluxation @ MCP and wrist, ulnar deviation @ MCP, radial deviation @ carpus, rheumatoid nodules, Heberden’s and Bouchard’s nodes Lumbar lordosis Gaitc Varus Valgus Genu recurvatum Baker cyst Gait3 Hand and wrist Knee Thumb flexion, extension, abduction, and adduction Finger flexion/extension Opposition Wrists flexion/extension Abduction (508) Adduction (208) Internal rotation (358) External rotation (458) Flexion (1208) Extension (20–308) Flexion (1358) Extension (108) Eversion (108) Inversion (108) ROM (Active and Passive) Abduction (1808) Adduction (508) Flexion (1808) Extension (608) Internal rotation (908) External rotation (908) Ankle and foot Dorsiflexion (208) Varus Plantarflexion (508) Valgus Subtalar joint— Achilles tendon inversion and Nails, bunion eversion (58) Hallux valgus Forefoot joints Metatarsus varus Joints of toes Pes planus Shoes a SEADS—Symmetry/swelling, Erythema, Atrophy, Deformity, and Surgeries/scars b SWAT—Swelling/synovitis, Warmth, Anatomic landmarks, Tenderness c Gait—heel strike, foot flat (mid-stance), heel off (lift off), toes off (swing) Hip Winging of scapulae Shoulder Inspection (SEADSa) Joint Examination Patella, tibial tuberosity Head of tibia/fibula Joint line tenderness Femoral condyles Bursas (suprapatellar, subpatellar, infrapatellar, anserine) Bulge test, balloon test, patella tap Achilles tendon Malleolus Anterior talofibular ligament Deltoid ligament Calcaneus Base of MTP Calcaneus Navicular Anterior drawer test Lateral/medial stability Subtalar complex stability Achilles tendon rupture Anterior drawer test, Lachman test, pivot shift (anterior cruciate ligament) Posterior drawer test (posterior cruciate ligament) Collateral ligaments McMurray test, medial–lateral grind test (meniscal) FABER test (groin pain=hip joint, buttock pain=SI joint) Thomas test (hip flexion contracture) Trendelenburg test (weakness of gluteus medius on standing side) Leg length discrepancy (true and false) Wrist Carpal joints MCP joints PIP joints DIP joints ASIS Iliac crest SI joint Greater trochanter Ischial tuberosity Special tests Initial abduction against resistance (supraspinatus) External rotation against resistance (infraspinatus and teres minor) Internal rotation against resistance (subscapularis) Relocation and anterior release tests (shoulder instability) Biceps load I and II (labrum tear) Biceps tendonitis Also examine C-spine and upper limb (neurological testing) Tinel’s test, Phalen’s test (carpel tunnel syndrome) Finkelstein’s test (de Quervain’s tenosynovitis) Hand grip strength and function (write) Neurological testing of hand Clavicle, AC joint, coracoid process, acromion, spine of scapula, greater and lesser tuberosity of humerus, biceps tendon Palpation (SWATb) 294 Joint Examination ... testing at 24 48 h TREATMENTS steroids and others (lesion dependent) 292 Approach to Serologies Approach to Serologies INFLAMMATORY MARKERS ERYTHROCYTE SEDIMENTATION RATE (ESR) (non specific) DISORDERS... positive mothers, cutaneous lupus rash, photosensitivity, and thrombocytopenia in SLE 293 Approach to Serologies SJOGREN’S SYNDROME (CONT’D) INFLAMMATORY MYOPATHIES (CONT’D) ANTI LA (SS B) DISORDERS... very useful for diagnosis of rheumatoid arthritis (sens 85%, spc 95%) For patients with elevated rheumatoid factor of >50 U/mL and fulfilling other criteria, rheumatoid arthritis is diag nosed without