Diagnosis and management of pulmonary vasculitis

Ther Adv Respir Dis. 2012 Dec;6(6):375-90. doi: 10.1177/1753465812454693. Epub 2012 Aug 9.


The pulmonary vasculitides are a heterogeneous group of disorders characterized pathologically by vascular destruction with cellular inflammation and necrosis. These disorders can affect small, medium, and large vessels and may be primary or occur secondary to a variety of conditions. Vasculitis involving the lungs is most commonly due to primary, idiopathic, small-vessel antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, which includes granulomatosis with polyangiitis (formerly Wegener's granulomatosis), Churg-Strauss syndrome, and microscopic polyangiitis. From a clinical perspective these remain among the most challenging of diseases both in terms of diagnosis and treatment. This review will focus on diagnosis and management of ANCA-associated vasculitides.

Publication types

  • Review

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / physiopathology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / therapy
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / physiopathology
  • Churg-Strauss Syndrome / therapy
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / physiopathology
  • Granulomatosis with Polyangiitis / therapy
  • Humans
  • Inflammation / etiology
  • Inflammation / physiopathology
  • Inflammation / therapy
  • Lung Diseases / diagnosis*
  • Lung Diseases / physiopathology
  • Lung Diseases / therapy
  • Microscopic Polyangiitis / diagnosis
  • Microscopic Polyangiitis / physiopathology
  • Microscopic Polyangiitis / therapy
  • Necrosis