Pathogenesis of Lung Vasculitis

Patrick H Nachman; Ashley G Henderson

doi:10.1055/s-0031-1279822

Seminars in Respiratory and Critical Care Medicine, Table of Contents

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

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

Abstract

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.

KEYWORDS

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

Full Text

References

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Patrick H NachmanM.D.

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

Chapel Hill, NC 27599

Email: patrick_nachman@med.unc.edu