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Wegener's granulomatosis
- Triad of necrotizing vasculitis, granulomas in the lungs and URT, and necrotizing glomerulonephritis
- Affects small vessels
- Patients may present with hemoptysis, hematuria, sinusitis
- c-ANCA and nodular densities on CXR
- Treat with cyclophosphamide
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Microscopic polyangitis
- Vasculitis of small vessels
- p-ANCA
- No granulomas
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Churg-Strauss
- Granulomatous vasculitis
- Can present with skin lesions and peripheral neuropathy (e.g. foot/wrist drop)
- Seen in asthmatics
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Sturge-Weber disease
- Congenital disorder of capillaries
- Port-wine stains on face
- Seizures
- Early-onset glaucoma
-
Henoch-Schonlein Purpura
- Common childhood vasculitis
- Presents with palpable purpura on buttocks and legs
- Triad of skin, joints, and GI tract
- Follows URIs
- Associated with IgA nephropathy
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Buerger's disease
- Segmental obliteration of small and medium blood vessel lumina
- Seen in smokers
- Intermittent claudication of vessels
- Classically involves lower extremities
- Inflammation extends from artery to vein and nerve
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Kawasaki's disease
- Necrotizing vasculitis of small vessels (often coronary vessels), seen in Asian children
- Fever, conjunctivitis, "strawberry tongue," lymphadenitis
- Often self-limiting
- Treat with IvIg and aspirin
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Polyarteritis Nodosa
- Immune complex mediated transmural vasculitis with fibrinoid necrosis
- Affects renal and visceral vessels
- Many patients are HBV positive (seen in IV drug users)
-
Takayasu's arteritis
- Granulomatous thickening of aortic arch and proximal great vessels
- Loss of pulse in upper extremities ("pulseless disease")
- Fever, myalgia, night sweats, skin nodules, ocular disturbances
- Affects young Asian women
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Temporal arteritis
- Most common vasculitis
- Affects medium and large arteries, typically the temporal artery
- Granulomatous inflammation
- Affects older women
- Symptoms include unilateral headache, jaw claudication, impaired vision
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Polymyalgia rheumatica
- Temporal arteritis with systemic involvement
- Presents with temporal arteritis, flu-like symptoms, and joint pain
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Raynaud's
- Episodic arterial vasospasm in the extremities
- Can be primary, or secondary to SLE, Buerger's, scleroderma, or CREST
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