Vasculitis is a general term for inflammation of blood vessel walls, resulting in ischemia of distal tissues. Most vasculitides are autoimmune diseases and the treatment involves immunosuppression or immunomodulation.
|| Takayasu arteritis. Primarily affects the aorta and its main branches. Patients are usually less than 50 years old. May be associated with anti-endothelial cell antibody.
Giant cell (temporal) arteritis. Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. Patients are usually over 50 years old.
Dermatological findings include:
| Polyarteritis nodosa. Systemic necrotizing vasculitis and aneurysm formation with sparing of the lungs and glomeruli. Blood tests for autoimmunity are usually normal; however, tests for inflammation (such as the erythrocyte sedimentation rate) may be abnormal.
Isolated central nervous system vasculitis. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy.
Associated with Type III, immediate hypersensitivity.
|| Granulomatous polyangiitis (Wegener's granulomatosis). Systemic vasculitis of medium and small arteries, including venules and arterioles. Produces granulomatous inflammation of the respiratory tracts and necrotizing, pauci-immune glomerulonephritis. Most common cause of saddle nose deformity in USA (nose flattened due to destruction of nasal septum by granulomatous inflammation). Almost all patients with Wegener's have c-ANCA, but not vice versa.
Churg-Strauss arteritis. Affects medium and small vessels with vascular and extravascular granulomatosis. Classically involves arteries of lungs and skin, but may be generalized. The triad asthma, eczema and renal abnormalities (e.g., red blood cell casts in urine) should raise suspicion, calling for an eosinophil count. Eosinophilia, with this clinical presentation, is grounds for a preliminary diagnosis, but immunologic confirmation is needed.
Microscopic polyarteritis/polyangiitis. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes granulomatous polyangiitis since both are associated with ANCA and similar extrapulmonary manifestations. Patients do not usually have symptomatic or histologic respiratory involvement.
|Treatment depends on whether the goal is to induce remission or maintenance and depends on severity of the vasculitis.|
|| Henoch-Schonlein purpura (HSP). Systemic vasculitis due to tissue deposition of IgA-containing immune complexes. This is the most common vasculitis in children.
Hypocomplementemic urticarial vasculitis
|Variable-vessel vasculitis (VVV)
|Single-organ vasculitis (SOV)
Cutaneous leukocytoclastic angiitisUsually due to a hypersensitivity reaction to a known drug. There is presence of skin vaculitis termed leukocytoclastic vasculitis with palpable petechiae or purpura. Most prominent in postcapillary venules.
|Vasculitis associated with systemic disease
|Vasculitis associated with probable etiology
Drug-associated immune complex vasculitis
Drug-associated ANCA-associated vasculitis
In general, treatment involves immunosuppression, usually starting with corticosteroids but often using cytotoxic agents such as cyclophosphamide or methotrexate as well. Plasmapheresis may be useful. Immunologic therapies are evolving.
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