Pathogenesis of lung vasculitis

Patrick H. Nachman, Ashley G. Henderson

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations

Abstract

Vasculitides that affect the lung represent a diverse group of diseases with various systemic clinical manifestations, and include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Churg-Strauss syndrome (CSS), and anti-glomerular basement membrane (anti-GBM) disease (Goodpasture syndrome). The etiologies of these diseases remain largely unknown. Although the pathogenic mechanisms of each differ, these diseases overlap by the presence of anti-neutrophil cytoplasmic autoantibodies in the vast majority of patients with MPA and GPA, and a substantial minority of patients with CSS and anti-GBM disease. This article reviews the current understanding of the pathogenesis of these four disease entities.

Original languageEnglish (US)
Pages (from-to)245-253
Number of pages9
JournalSeminars in Respiratory and Critical Care Medicine
Volume32
Issue number3
DOIs
StatePublished - Jun 22 2011
Externally publishedYes

Keywords

  • Churg Strauss syndrome
  • Goodpasture syndrome
  • Vasculitis
  • Wegener granulomatosis
  • anti-GBM
  • microscopic polyangiitis
  • pathogenesis

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