Flashcards in Vasculitis Deck (19):
2 types of large vessel vasculitis
GCA and Takayasu
2 types of medium vessel vasculitis
POlyarteritis nodosa and Kawasaki Disease
2 categories of small vessel vasculitis
ANCA-associated vasculitis and Immune complex vasculitis
3 types of ANCA-associated vasculitis and type of ANCA
Microscopic polyangitis: pANCA, MPO
GPA: cANCA, PR3
Eosinophilic granulomatosis w/ polyangiitis: either ANCA
4 types of immune complex vasculitis
cryoglobulinemic vasculitis, anti-glomerular BM disease (Goodpasture), IgA vasculitis, hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Treating small / medium vessel vasculitis
High dose corticosteroids and immune suppressive meds (azathriprine, Mtx, mycophenolate mofetil
Treating large vessel vasculitis
treating life threatening / organ-threatening vasculitis
High dose steroids + cyclophosphamide or rituximab
Giant Cell Arteritis
•By far the most common systemic vasculitis
•Sxs – new headache, constitutional sxs, proximal myalgia, painful shoulder / hip movement, jaw claudication, scalp tenderness, visual sxs (may cause blindness)
•Associated w/ polymyalgia rheumatica (50%)
•Look for dilation of temporal artery and loss of pulse.
•Diagnosis – obtain biopsy as soon as possible
• Shows granulomatous or mononuclear infiltrate in artery w/ fragmentation of internal elastic lamina.
•Treatment – begin high dose steroids right away if suspected.
•9x more common in women.
•Avg age of onset under 40.
•Clinical manifestations – claudication of extremities, “pulseless” (esp in upper extremities), bruit over subclavian artery / abdominal aorta, abnormal angiogram / MRA
•Treat w/ high dose steroids
Polyarteritis Nodosa (PAN)
4 affected organ systems
•Skin – purpura, livedo reticularis
•Myalgia / arthralgia
•Nerve – mononeuropathy / multiple mononeuropathies
•Kidney – HTN, elevated Cr, proteinuria
•Associated w/ Hep B
•Diagnosis – biopsy or angiography (shows focal / segmental vasculitis)
•Treatment – steroids, cyclophosphamide if severe. Use steroids + anti-viral if pos for Hep B.
•Clinical manifestations – palpable purpura, arthralgias, LFTs, elevated Cr, proteinuria, low C4 / RF, cryoglobulins
•Associated w/ Hep C (>90%)
•Diagnose w/ biopsy
•Treatment – steroids + anti-viral +/- plasmapheresis. Use rituximab for severe cases.
•Major Ag: serine protease PR3
•Major Ag: myeloperoxidase (MPO)
•Diseases: microscopic polyangiitis, Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss), IBD, chronic liver disease, HIV, lupus, etc.
•Clinical manifestations – sinusitis, epistaxis, otitis media, anemia of chronic disease, RBC casts
• Lungs: infiltrates, nodules, cavitary lesions → hemoptysis, dyspnea,
• Kidney: HTN, pauci-immune glomerulonephritis, kidney failure
•Labs: cANCA, PR3, elevated ESR / Cr
•Treatment – high dose prednisone + (cyclophosphamide or rituximab)
Microscopic Polyangiitis (MPA)
•Clinical manifestations – pulmonary infiltrates, proteinuria, hematuria, RBC casts, pANCA, MPO. Usually no lung involvement as w/ GPA.
•Biopsy shows pauci-immune glomerulonephritis and necrotizing vasculitis
•Treatment – steroids → cyclophosphamide or rituximab
Eosinophilic granulomatosis w/ polyangiitis (EGPA, Churg-Strauss)
Clinical manifestations (8)
•Clinical manifestations – asthma, allergic rhinitis, nasal polyposis, eosinophilia, non-fixed pulmonary infiltrates, cardiomyopathy, MI, pANCA
•Biopsy shows extravascular eosinophils
•Treatment – steroids → cyclophsophamide
•More common in men from Mediterranean and Far East. Onset 2nd-3rd decade.
•Chronic vasculitis of arteries and veins of all sizes. Large vessel disease is more rare but more serious. Affects veins more than other diseases.
•Clinical manifestations – recurrent painful ORAL / SCROTAL ULCERS, erythema nodosum (panniculitis of small vessels), superficial / deep vein thrombosus, uveitis / retinal vasculitis → blindness, bowel disease like Crohn’s, aseptic meningitis, meningoencephalitis.
•Prognosis – increased mortality, esp in young men. Mortality mainly from large vessel disease, esp bleeding pulmonary artery / GI aneurysms and CNS involvement.
• Local corticosteroids / cochicine for mucocutaneous ulcers
• Azathioprine for eye involvement