Microscopic Polyangiitis

Sharon A. Chung, Philip Seo

Research output: Contribution to journalReview article

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.

Original languageEnglish (US)
Pages (from-to)545-558
Number of pages14
JournalRheumatic Disease Clinics of North America
Volume36
Issue number3
DOIs
StatePublished - Aug 1 2010

Keywords

  • Antineutrophil cytoplasmic autoantibodies
  • Microscopic polyangiitis
  • Pulmonary-renal syndrome
  • Vasculitis

ASJC Scopus subject areas

  • Rheumatology

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