Microscopic polyangiitis.
In 1923, Friedrich Wohlwill described two patients with a "microscopic form of periarteritis nodosa," which was distinct from the classical form. This disease, now known as microscopic polyangiitis (MPA), is a primary systemic vasculitis characterized by inflammation of the small-caliber blood vessels and the presence of circulating antineutrophil cytoplasmic antibodies. Typically, microscopic polyangiitis presents with glomerulonephritis and pulmonary capillaritis, although involvement of the skin, nerves, and gastrointestinal tract is not uncommon. Treatment of MPA generally requires use of a cytotoxic agent (such as cyclophosphamide) in addition to high-dose glucocorticoids. Recent research has focused on identifying alternate treatment strategies that minimize or eliminate exposure to cytotoxic agents. This article reviews the history, pathogenesis, clinical manifestations, and treatment of MPA.
Chung
Sharon A
SA
Division of Rheumatology, Department of Medicine, Rosalind Russell Medical Research Center for Arthritis, University of California, San Francisco, CA, USA.
Seo
Philip
P
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D000963:Antimetabolites / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004334:Drug Administration Schedule; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D055953:Microscopic Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology*; D012074:Remission Induction
D019268:Antibodies, Antineutrophil Cytoplasmic; D000963:Antimetabolites; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide