Vasculitis Flashcards
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
corticosteroids
treating life threatening / organ-threatening vasculitis
High dose steroids + cyclophosphamide or rituximab
Giant Cell Arteritis Age How common? Sxs Association Clinical clues Diagnosis Treatment
- Age >50
- 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.
Takayasu's Arteritis Gender Age Clinical manifestations Treatment
- 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 Associated diseases Diagnosis Treatment
- 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.
Cryoglobulinemic vasculitis Clinical manifestations Associated disease Diagnosis Treatment
- 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.
cANCA
Major Ag
Disease
Appearance
- Major Ag: serine protease PR3
- Disease: GPA
- Granular staining
pANCA
Major Ag
Diseases (6)
- Major Ag: myeloperoxidase (MPO)
- Diseases: microscopic polyangiitis, Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss), IBD, chronic liver disease, HIV, lupus, etc.
GPA
Clinical manifestations
Labs
Treatment
- 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
Biopsy
Treatment
- 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)
Biopsy
Treatment
- Clinical manifestations – asthma, allergic rhinitis, nasal polyposis, eosinophilia, non-fixed pulmonary infiltrates, cardiomyopathy, MI, pANCA
- Biopsy shows extravascular eosinophils
- Treatment – steroids → cyclophsophamide
Behcet's Disease Epidemiology Pathology Clinical manifestations Prognosis Treatment
- 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.
- Treatment
- Local corticosteroids / cochicine for mucocutaneous ulcers
- Azathioprine for eye involvement
Polymyalgia Rheumatica Age Heritage Clinical manifestations Association Prognosis Treatment
- Avg age 60-80.
- More common in Northern European heritage.
- Clinical manifestations – pain / stiffness of neck, shoulder girdle, and pelvic girdle (symmetric). Malaise, fever, weight loss.
- Associated w/ GCA.
- Prognosis – no increase in mortality. Usually remits in 2-3 years.
- Treatment – extremely responsive to low dose prednisone. Monitor ESR / CRP. Educate regarding risk for GCA.
