Semin Respir Crit Care Med 2011; 32(3): 245-253
DOI: 10.1055/s-0031-1279822
© Thieme Medical Publishers

Pathogenesis of Lung Vasculitis

Patrick H. Nachman1 , Ashley G. Henderson2
  • 1University of North Carolina Kidney Center, Chapel Hill, North Carolina
  • 2Division of Pulmonary and Critical Care Medicine, School of Medicine, University of North Carolina, Chapel Hill, North Carolina
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Publication History

Publication Date:
14 June 2011 (online)

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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.

REFERENCES

Patrick H NachmanM.D. 

UNC Kidney Center, Campus Box 7155, University of North Carolina

Chapel Hill, NC 27599

Email: patrick_nachman@med.unc.edu