Journal
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
Volume 36, Issue 3, Pages 545-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.rdc.2010.04.003
Keywords
Microscopic polyangiitis; Vasculitis; Antineutrophil cytoplasmic autoantibodies; Pulmonary-renal syndrome
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Funding
- National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases [1K23AR052820-01]
- American College of Rheumatology Physician Scientist Development Award
- National Institutes of Health/National Center for Research Resources [5 KL2 RR024130-04]
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In 1923, Friedrich Wohlwill described two patients with a microscopic form of penarteritis 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.
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