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Rheum Dis Clin North Am. 2010 Aug;36(3):545-58. doi: 10.1016/j.rdc.2010.04.003. Epub 2010 Jun 11.

Microscopic polyangiitis.

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  • 1Division of Rheumatology, Department of Medicine, Rosalind Russell Medical Research Center for Arthritis, University of California, San Francisco, CA, USA.

Abstract

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.

Copyright 2010 Elsevier Inc. All rights reserved.

PMID:
20688249
[PubMed - indexed for MEDLINE]
PMCID:
PMC2917831
Free PMC Article

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