Vascullitic disorders

Disorder	Vasculitis	Epidemiology/Etiology	Clinical/Laboratory Findings
Takayasu's arteritis ("pulseless disease")	Granulomatous large vessel vasculitis involving aortic arch vessels	Young Asian women and children	Absent upper extremity pulse Visual defects, stroke
Giant cell (temporal) arteritis	Granulomatous large vessel vasculitis involving superficial temporal and ophthalmic arteries; thrombi contain microabscesses	Adults > 50 years of age	Temporal headache, jaw claudication (pain when chewing) Blindness on ipsilateral side Polymyalgia rheumatica (muscle and joint pain; normal serum creatine kinase) Increased ESR
Polyarteritis nodosa	Necrotizing medium-sized vessel vasculitis involving renal, coronary, mesenteric arteries (spares pulmonary arteries)	Middle-aged men Association with HBsAg (30%)	Vessels at all stages of acute and chronic inflammation Focal vasculitis produces aneurysms (detected with angiography) Organ infarction in kidneys (renal failure), heart (acute MI), bowels (bloody diarrhea), skin (ischemic ulcer)
Kawasaki disease	Necrotizing medium-sized vessel vasculitis involving coronary arteries (e.g., thrombosis, aneurysms)	Children < 4 years of age	Desquamating rash, swelling of hands and feet, cervical adenopathy, oral erythema Abnormal ECG (e.g., acute MI) Corticosteroids contraindicated (danger of vessel rupture)
Thromboangiitis obliterans (Buerger's disease)	Medium-sized vessel vasculitis with digital vessel thrombosis	Men 25-50 years of age who smoke cigarettes	Foot claudication, Raynaud's phenomenon, ulceration, gangrene
Raynaud's disease	Medium-sized vessel vasculitis involving digital vessels in fingers and toes	Young women Exaggerated vasomotor response to cold or stress	Paroxysmal digital color changes (white-blue-red sequence) Ulceration and gangrene in chronic cases
Raynaud's phenomenon	Medium-sized vessel vasculitis involving digital vessels in fingers and toes	Adult men and women Secondary to other diseases (e.g., systemic sclerosis, CREST syndrome)	Systemic sclerosis and CREST syndrome: digital vasculitis with vessel fibrosis, dystrophic calcification, ulceration, gangrene
Wegener's granulomatosis	Necrotizing medium-sized and small vessel vasculitis involving upper respiratory tract, lung, renal vessels	Childhood to middle age	Necrotizing vasculitis in upper respiratory tract (nasopharynx, sinuses, trachea), lower respiratory tract (pulmonary vessels; infarction, pneumonia), kidneys (crescentic glomerulonephritis)
			Necrotizing granulomas in upper respiratory tract (saddle nose deformity), lungs c-ANCA antibodies (>90% of cases) Treatment: corticosteroids, cyclophosphamide
Microscopic polyangiitis	Small vessel vasculitis involving skin, lung, brain, GI tract, and postcapillary venules and glomerular capillaries	Children and adults Precipitated by drugs (e.g., penicillin), infections (e.g., streptococci), immune disorders (e.g., SLE)	Vessels at same stage of inflammation Palpable purpura, glomerulonephritis p-ANCA antibodies (>80% of cases)
Churg-Strauss syndrome	Small vessel vasculitis involving skin, lung, heart vessels	Children and adults	Allergic rhinitis, asthma p-ANCA antibodies (70% of cases), eosinophilia
Henoch-Schönlein purpura	Small vessel vasculitis involving skin, GI tract, renal, joint vessels	Children and young adults Most common vasculitis in children IgA immunocomplexes	Palpable purpura of buttocks and lower extremities Polyarthritis, glomerulonephritis, GI bleeding
Cryoglobulinemia	Small vessel vasculitis involving skin, GI tract, renal vessels	Adults Association with HCV, type I MPGN	Cryoglobulins: immunoglobulins that gel at cold temperatures Palpable purpura, acral cyanosis of nose and ears and Raynaud's phenomenon (reverses when in warm room)
Serum sickness	Small vessel vasculitis involving immunocomplex deposition in skin vessels	Children and adults Complication of treatment of rattlesnake envenomation with horse-or sheep-based antivenin	Fever, urticaria with vasculitis, arthralgia, GI pain with melena
Infectious vasculitis	Small vessel vasculitis involving skin vessels	Children and adults Involves all microbial pathogens	Rocky Mountain spotted fever: tick transmission of Rickettsia rickettsiae; organisms invade endothelial cells; petechiae on palms spread to trunk Disseminated meningococcemia due to Neisseria meningitidis Capillary thrombosis produces hemorrhage into skin and confluent ecchymoses