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Microscopic polyangiitis is a pauci-immune nongranulomatous necrotizing vasculitis that (1) affects small blood vessels (capillaries, venules, or arterioles), (2) often causes glomerulonephritis and pulmonary capillaritis, and (3) is often associated with ANCA on immunofluorescence testing (directed against MPO, a constituent…
Primary angiitis of the CNS is a syndrome with several possible causes that produces small- and medium-sized vasculitis limited to the brain and spinal cord. Biopsy-proved cases have predominated in men who have a history of weeks to months of…
Named after the Turkish dermatologist who first described it, Behçet disease is of unknown cause and most commonly occurs in persons of Asian, Turkish, or Middle Eastern background. The protean manifestations are believed to result from vasculitis that may involve…
This disease is characterized by inflammatory destructive lesions of cartilaginous structures, principally the ears, nose, trachea, and larynx. Nearly 40% of cases are associated with another disease, especially other immunologic disorders (ANCA vasculitis, SLE, rheumatoid arthritis, or Hashimoto thyroiditis), cancers…
IgA vasculitis (Henoch-Schönlein purpura), the most common systemic vasculitis in children, occurs in adults as well. Typical clinical features are palpable purpura, arthritis, and hematuria. Abdominal pain occurs less frequently in adults than in children. Pathologic features on skin biopsy…
Cryoglobulinemia can be associated with an immune-complex–mediated, small-vessel vasculitis. Chronic infection with hepatitis C is the most common underlying condition; cryoglobulinemic vasculitis also can occur with other chronic infections (such as subacute bacterial endocarditis, osteomyelitis, HIV, and hepatitis B), with…
Exposure to levamisole, a prevalent adulterant of illicit cocaine in North America, can induce a distinctive clinical syndrome of retiform purpura and cutaneous necrosis affecting the extremities, ears, and skin overlying the zygomatic arch. The syndrome has been linked to…
Eosinophilic granulomatosis with polyangiitis (previously called Churg-Strauss syndrome) is an ANCA-associated vasculitis (along with granulomatosis with polyangiitis and microscopic polyangiitis), although the presence of ANCA occurs in < 50% of patients (usually anti-MPO). It is characterized by peripheral eosinophilia, sinusitis…
Granulomatosis with polyangiitis (formerly Wegener granulomatosis), which has an estimated incidence of approximately 12 cases per million individuals per year, is one of three vasculitides associated with ANCA. The other “ANCA-associated vasculitides” include microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.…
Polyarteritis nodosa is a necrotizing arteritis of medium-sized vessels that has a predilection for involving the skin, peripheral nerves, mesenteric vessels (including renal arteries), heart, and brain but spares the lungs. Polyarteritis nodosa is rare, with a prevalence of 30…