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Wegener's Granulomatosis

Lung and upper airway necrotiing granulomas, glomerulonephritis, necrotizing vasculitis. Hemoptysis, hematuria, otitis media, mastoiditis, cough dyspnea. c-ANCA positive. treat with cyclophosphamide and corticosteroids. Childhood to middle age.

Sturge-Weber

Congenital vascular disorder affecting capillary blood vessels. Port-wine stain on face, ipsilateral intracerebral AVM, seizures, early onset glaucoma.

Henoch-Schonlein purpura

most common childhood vasculitis. Skin rash on buttocks and legs - palpable purpura, arthrlagia, abdominal pain, melena with intestinal hemorrhage. Following URI's. IgA immune complex mediated. Affects 1. Skin 2. Joints 3. GI

Buerger's disease

thrombosing/infarction of peripheral medium-vessels. Seen in heavy smokers. Nodular phlebitis, claudication, Raynaud's, gangrene and finger amputation.

Kawasaki's

Acute, self limiting necrotizing vasculitis in infants/children. Associated with asians. Fever, coronary aneurysms, erythema, edema of hands and feet, cervical adenopathy, oral erythema and cracking of lips. Small and medium vessel.

Takayasu's arteritis

"Pulseless disease" granulomatous thickening of aortic arch and proximal great vessels. Asian females over 40. absent upper extremity pulse, visual defects, stroke, fever, arthritis, night sweats. Medium and large arteries.

Temporal arteritis

Most common vasculitis affecting medium and large arteries. Unilateral headache, jaw claudication, impaired vision (occlusion of opthalmic artery can lead to blindness). Increased ESR. Associated with polymalgia rheumatica. Granulomatous inflammation in elderly females

Polyarteritis nodosa

Immune complex-mediated, transmural vasculitis with fibrinoid necrosis. Fever weight loss, malaise, abdominal pain, melena, headache, neurologic dysfunction, cutaneous eruptions. Hepatitis B in 30% of patients. Multiple aneurysms. Small and medium arteries. Can effect any organ except for lung - typically renal and visceral vessels. Young people

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