Pathogenesis of ANCA-Associated Pulmonary Vasculitis

Marco A. Alba; J. Charles Jennette; Ronald J. Falk

doi:10.1055/s-0038-1673386

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

Semin Respir Crit Care Med 2018; 39(04): 413-424
DOI: 10.1055/s-0038-1673386

Review Article

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pathogenesis of ANCA-Associated Pulmonary Vasculitis

Autoren

Marco A. Alba

¹Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
J. Charles Jennette

¹Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
Ronald J. Falk

²University of North Carolina Kidney Center, Chapel Hill, North Carolina

³UNC Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina

Abstract

Antineutrophil cytoplasmic antibodies (ANCAs) are autoantibodies specific for antigens located in the cytoplasmic granules of neutrophils and lysosomes of monocytes. ANCAs are associated with a spectrum of necrotizing vasculitis that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Pulmonary vasculitis and related extravascular inflammation and fibrosis are frequent components of ANCA vasculitis. In this review, we detail the factors that have been associated with the origin of the ANCA autoimmune response and summarize the most relevant clinical observations, in vitro evidence, and animal studies strongly indicating the pathogenic potential of ANCA. In addition, we describe the putative sequence of pathogenic mechanisms driven by ANCA-induced activation of neutrophils that result in small vessel necrotizing vasculitis and extravascular granulomatous necrotizing inflammation.

Keywords

ANCA - granulomatosis with polyangiitis - microscopic polyangiitis - pulmonary vasculitis

Volltext

Referenzen

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