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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

Marco A. Alba
1   Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
,
J. Charles Jennette
1   Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
,
Ronald J. Falk
2   University of North Carolina Kidney Center, Chapel Hill, North Carolina
3   UNC Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
› Author Affiliations
Further Information

Publication History

Publication Date:
07 November 2018 (online)

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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

Note

M.A. Alba was supported by Consejo Nacional de Ciencia y Tecnología (CONACyT), Mexico.


 

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