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.
© Thieme Medical Publishers.
MeSH terms
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Anti-Glomerular Basement Membrane Disease / etiology
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Anti-Glomerular Basement Membrane Disease / immunology
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Anti-Glomerular Basement Membrane Disease / pathology
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Antibodies, Antineutrophil Cytoplasmic / analysis
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Antibodies, Antineutrophil Cytoplasmic / immunology*
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Churg-Strauss Syndrome / etiology
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Churg-Strauss Syndrome / immunology
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Churg-Strauss Syndrome / pathology
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Granulomatosis with Polyangiitis / etiology
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Granulomatosis with Polyangiitis / immunology
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Granulomatosis with Polyangiitis / pathology
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Hemorrhage / etiology
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Hemorrhage / immunology
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Hemorrhage / pathology
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Humans
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Lung Diseases / etiology
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Lung Diseases / immunology*
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Lung Diseases / pathology
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Microscopic Polyangiitis / etiology
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Microscopic Polyangiitis / immunology
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Microscopic Polyangiitis / pathology
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Vasculitis / complications
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Vasculitis / etiology
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Vasculitis / immunology*
Substances
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Antibodies, Antineutrophil Cytoplasmic