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- Patrick H Nachman and Ashley G Henderson.
- University of North Carolina Kidney Center, Chapel Hill. patrick_nachman@med.unc.edu
- Semin Respir Crit Care Med. 2011 Jun 1;32(3):245-53.
AbstractVasculitides 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.© Thieme Medical Publishers.
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