McGowan Flashcards
what titer level of ANA is normal/negative
-<1:40 . .. so higher ration like 1:160 is +
Homogenous ANA staining
Drug induced SLE
Speckled staining pattern of ANA
Sjogren’s
Centromere ANA staining pattern
-Limited systemic sclerosis (CREST)
Nucleolar ANA staining pattern
-Diffuse Systemic sclerosis
multisystem involvement of SLE
- butterfly rash
- Pleural effusions
- Heart problems
- Lupus nephritis
- arthritis
- Raynaud’s
Serology of SLE?
which correlates with disease activity
- ANA
- Anti-ds DNA (DISEASE ACTIVITY)
- Sm
- low C3 and C4: increased consumptions
SLE and anti-phospholipid antibody syndrome (APS)
- 1/3 of SLE pts have APS
- treat with anticoagulation
what are the 3 types of antiphospholipid antibodies
- type 1: causes biologic false + syphilis test
- type 2: Lupus anticoagulant- risk factor for venous and arterial thrombosis and miscarriage, prolongs aPTT, presence confirmed by an abnormal Russel viper venom time (RVVT)
- Type 3: anti cardiolipin antibodies (beta2GPI)
Cotton wool spots
SLE/APS retinopathy
serology for drug induced lupus like syndrome
- ANA
- Anti-histone antibodies
Neonatal lupus affects children born of mothers with what Abs
- Ro (SSA)
- La (SSB)
major complication of Neonatal Lupus
-permanent complete heart block
Treatment of SLE
- Avoid sun exposure, wear sunscreen
- NSAIDs
- Corticosterioids
- Hydroxychloroquine
what type of hypersensivity is SLE
- type 3
- immune complex mediated
hallmark of Scleroderma (systemic sclerosis)
- thickening and hardening of the skin
- microangiopathy and fibrosis of the skin and visceral organs
3 types of scleroderma
- Diffuse
- Limited (CREST: calcinosis cutis, Raynauds secondary, esophageal dysmotility, sclerodactyly, telangiectasia)
- Localized
Serology for Diffuse scleroderma
- Anti-Scl 70 aka anti DNA topoisomerase I
- Anti-RNA polymerase III
Serology for Limited cutaneous scleroderma
-Anti-centromere
GI effects of scleroderma
- malnutrition: fat, protein, B12, and vitamin D deficiency
- GERD –>Barrett Esophagus –> increased risk of esophageal adenocarcinoma
- GAVE aka watermelon stomach
pulmonary effects of scleroderma
- primary cause of morbifity and mortality
- interstitial lung disease: diffuse . .dry Velcro crackles
- Pulmonary artery HTN: limited
- right heart cath to confirm dx
- increased incidence of bronchoalveolar carcinoma
Renal effects of scleroderma
- Chronic kidney disease
- Renal crisis uncommon but life threatening: abrupt onset of malignant HTN, hemolytic anemia, and progressive renal insufficiency
- more common in DIFFUSE scleroderma
- Treatment: avoid high dose corticosteroids
MSK and other effects of scleroderma
- Carpal tunnel syndrome
- Hypothyroid from thyroid fibrosis
highlights for Sjogren
- Sicca symptoms (immune mediated dysfunction of lacrimal and salivary glands
- Keratoconjunctivitis sicca (foreign body sensation-inadequate tear production)
- Strone association with B cell non Hogkin lymphoma
Lip biopsy in Sjogren
- characteristic lymphoid foci in accessory salivary glands
- essential for diagnosis
Serology for Sjogren-
- ANA
- RF
- Hypergammaglobulinemia
- anti SSA/Ro: may lead to newborn complete heart block
- Anti SSB/La: never present without Ro
Highlights for inflammatory myopathies
- Symmetrical bilateral proximal muscle weakness: difficulty rising from chair or bathtub or climbing stairs
- elevated CK and aldolase
- characteristic muscle biopsy
Highlights for Dermatomyositis
- Heliotrope rash
- “shawl sign”: erythema over neck/shoulders, upper chest and back
- increased risk of occult MALIGNANCY
- Dx: biopsy-perimysial and perivascular inflammation . . Perifascicular atrophy
serology for Dermatomyositis
- Elevated CK, aldolase
- Anti Jo-1, anti-Mi2, and anti-MDA5, anti-P155/140
Malignancies and Dermatomyositis
- Ovarian most common
- check transvaginal US, CT abd/pelvis, CA-125
highlights for polymyositis
- NO skin changes like Dermatomyositis
- elevated CK
- Anti-Jo-1
- muscle bx: endomysial inflammation
management of dermatomyositis and polymyositis
Corticosteroids
highlights for inclusion body myositis
- Males more
- Finger flexion and quad weakness
- CK is mild elevation or normal
- Muscle bx: endomysial inflammation, RIMMED VACUOLES, invasion of non-necrotic muscle fibers, ANTI-cN1A autoantibodies
highlights for Takayasu Arteritis
- Asian less than 40
- Large vessel
- long smooth tapered stenosis
- “pulseless disease”: obliterate UE peripheral pulses, collaterals so limb loss from ischemia is rare
- Diagnosis: MRI or CT angiography
- Histology: granuloma with some giant cells
- Tx: Glucocorticoids
highlights for Behcet Syndrome
- Silk route: Turkey, Asia, Mid East
- HLA-B51
- Triad: recurrent mouth ulcers, genital ulcers, eye inflammation (uveitis)
- Large vessel=aneurysm
- Venous involvement: DVT
- Pathergy: pustules at site of sterile needle pricks
- 50% pulmonary involvement
- Retinopathies
highlights for Polyarteritis Nodosa
- associated with HBV
- males more
- 80% have vasculitis neuropathy . .foot drop
- LUNGS SPARED
- Bx: infiltration and destruction of blood vessels by inflammatory cells–>fibrinoid necrosis, NO granulomas
- Angiogram: micro aneurysm
- ANCA negative, check HBsAg and HBeAg
highlights for Kawasaki
- <5yr
- Asian
- “mucocutaneous lymph node syndrome”
- “strawberry tongue”
- Death: from coronary involvement (aneurysm or MI-can occur years later)
- Tx: IVIG w/in 10 days of sxs and high dose ASA . . YES ASPIRIN in a pediatric pts
highlights for Granulomatosis with polyangiitis: aka Wegener’s Granulomatosis
- C-ANCA aka PR3-ANCA
- respiratory tract (Saddle nose)
- Chest XR: cavitary lesions
- kidney involvement
- Hallmarks: Granulomatous inflammation, Necrotizing vasculitis, Segmental Glomerulonephritis
highlights for Eosinophilic Granulomatosis with polyangiitis aka Churg-Strauss Syndrome
- ANCA + in 50% . .typically MPO-ANCA
- Granulomas with eosinophilia
- Hallmarks: asthma + eosinophilia
Highlights for Thromboangiitis Obliterans aka Buerger Disease
- young males <35
- only occurs in smokers
- Dx: angiography-“corkscrew” appearance
- Tx: STOP SMOKING
Secondary Raynaud
- more severe than primary . .ischemia
- unilateral (primary is bilateral)
- Nailfold Capillaroscopy: distorted with widened and irregular loops, dilated lumen and areas of vascular “dropout” . . normal in primary
what frequently occurs with polymyalgia Rheumatica
Giant cell arteritis aka Temporal Arteritis
highlight for Giant Cell arteritis aka Temporal Arteritis
- Cranial arteries (temporal/facial/ophthalmic)
- Aortic arch
- Headache, Jaw claudication, visual abnormalities
- super high ESR . . >50 mm/h
- HLA-DR4
- associated with polymyalgia rheumatic
- Temporal artery bx (gold standard for dx: need a 1 cm segment)
- multinucleated giant cells
- Start corticosterioids before biopsy .. if no treatment then blindness
highlights for polymyalgia Rheumatica
- Associated with giant cell arteritis
- Stiffness, soreness and pain
- weakness as result of pain
- NO inflammation on muscle bx
- muscle enzymes and EMG normal
- elevated ESR and CRP
