Vasculitides

Vasculitis
Vacsulitis is an inflammation of the vessel wall. Inflammation results from immune complex deposition or from cellmediated immune reactions directed against the vessel wall. It can involve small, medium and large blood vessels.

Categorized by vessel size

Small
Wegeners granulomatosis Churg-Strauss syndrome Microscopic Polyangiitis Primary pauci-immune crescentic glomerulonephritis : Limited to kidneys and paucity of antibodies Sturge-Weber disease Henoch-Schonlein purpura

Small and Medium

Buergers disease Kawasaki disease Polyarteritis nodosa

Medium and Large

Takayasus arteritis Temoral (giant cell artiritis)

Wegeners Granulomatosis
Necrotizing granulomatous vasculitis of the lungs and upper airways, Focal necrotizing glomerulonephritis. Goodpastures syndrome is a mimic Hemoptysis, hematuria, perforation of nasal septum, sinusitis, otitis media c-ANCA, Chest X-ray may reveal nodular densities Tx: Cyclophospamide and corticosteroids

Microscopic Polyangiitis
Symptoms: skin nodules, hemoptysis, abdominal pain, hematuria Glomerulonephritis in 90%. Often an immunologic reaction to drug (penicillin) and microorganisms. Like Wegeners but lacks granulomas p-ANCA

Churg-Strauss Syndrome
Granulomatous vasculitis with eisonophilia Asthma and allergitic rhinitis Skin lesions and peripheral neuropathy (wrist/foot drop) May involve heart, GI, and kidneys p-ANCA positive

Sturge-Weber disease
Port-wine stain on face, leptomeningeal angiomatosis (intracerebral AVM), seizures, and early onset glaucoma

Henoch Schonlein purpura

Palpable purpura on buttocks and legs, arthralgia, and abdominal pain Follows URIs IgA immune complexes

Buergers disease
Also known as thromboangiitis obliterans Classic presentation: 40yo male smoker presents with intermittent claudication Other symptoms: Phlebitis, Raynauds May lead to gangrene and autoamputation of digits

Kawasaki Disease
Asian kiddos Acute and self limiting Fever, conjunctivitis, strawberry tongue, lymphadenitis, rash, and 20 % involve coronary artery vasculitis leading to aneurysms and thrombosis due to stasis Tx: Aspirin (Reyes is a better evil ???) and IVIG

Polyarteritis Nodosa

Immune complex mediated transmural vasculitis with fibrinoid necrosis Segmental necrotizing inflammation causes aneurysms and thrombosis Symptoms: malaise, fever, weight loss, hypertension, abdominal pain and melena, muscular pain, peripheral neuritis, renal failure. Typically involves renal and visceral vessels and not pulmonary arteries. 30 % of pts are Hep-B seropositive. No association with ANCA Tx: Corticosteroids, Cyclophosphamide

Takayasu Arteritis (Pulseless disease)

Asian women <40 years of age Weakened pulses in arms. Fever, arthritis, Night sweats, Myalgia, Skin nodules, ocular disturbances Granulomatous thickening of aortic arch and/or proximal great vessels. Associated with elevated ESR

Giant Cell Arteritis

Most common of the vasculitides Focal thickening with granulomatous inflammation primarily in temporal, vertebral, ophthalmic (carotid branches). Elevated ESR Mostly in females > 50 years of age Unilateral headache, jaw claudication, impaired vision (ophthalmic artery occlusion possibly leading to irreversible blindness) 50 % have coexisting polymyalgia rheumatica (PMR) Responds to anti-inflammatory therapy