Vasculitis: Microscopic Polyangiitis

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Microscopic polyangiitis (MPA) is a rare disease of unknown etiology. It is characterized by necrotizing inflammation of small blood vessels (arterioles, capillaries, and venules) in the absence of immune deposits and granulomatous inflammation. Antibodies against the content of azurophilic granules in neutrophilic granulocytes (antineutrophil cytoplasmic antibodies (ANCA)), most commonly targeting myeloperoxidase (MPO-ANCA), are present in the majority of patients. Although the etiology of MPA remains unknown, clinical observations and animal models suggest a causal association with MPO-ANCA. Clinically, MPA commonly presents with systemic symptoms, palpable purpura, renal dysfunction, pulmonary hemorrhage, and mononeuritis multiplex. If larger arteries are involved, patients may display disease manifestations historically attributed to panarteritis nodosa. Rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage represent the most frequent life-threatening complications. The combination of glucocorticoids and cyclophosphamide is typically used for remission induction therapy. Despite aggressive therapy, relapses occur commonly and treatment-associated morbidity and mortality are frequently encountered.

Original languageEnglish (US)
Title of host publicationEncyclopedia of Respiratory Medicine, Four-Volume Set
PublisherElsevier Inc.
Pages422-428
Number of pages7
ISBN (Print)9780123708793
DOIs
StatePublished - Jan 1 2006

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Keywords

  • ANCA-associated vasculitis
  • Antineutrophil cytoplasmic antibodies (ANCA)
  • Cyclophosphamide
  • Diffuse alveolar hemorrhage
  • Glucocorticoids
  • Leukocytoclastic vasculitis
  • Microscopic polyangiitis
  • Myeloperoxidase
  • Palpable purpura
  • Pauci-immune focal segmental necrotizing glomerulonephritis
  • Pulmonary-renal syndrome

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Specks, U., & Peikert, T. D. (2006). Vasculitis: Microscopic Polyangiitis. In Encyclopedia of Respiratory Medicine, Four-Volume Set (pp. 422-428). Elsevier Inc.. https://doi.org/10.1016/B0-12-370879-6/00423-3