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Pathogenesis of ANCA-Associated Vasculitis

  • VASCULITIS (LR ESPINOZA, SECTION EDITOR)
  • Published:
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Abstract

Antineutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitides (AAV) are a group of systemic vasculitis syndromes characterized by inflammation and necrosis of blood vessel walls. Genetic, epigenetic, and environmental factors contribute to the etiology and pathogenesis of AAV. On the basis of currently available clinical and experimental evidence, it is reasonable to believe that, in predisposed patients, different triggers can lead to the production of autoantibodies (ANCA) that, in the context of an inflammatory environment, can cause tissue inflammation and vascular injury. Several different pathways and mechanisms in the pathogenesis of AAV are described in this contemporary review.

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Abbreviations

NG:

Necrotizing granuloma

H:

Histiocyte

PMN:

Polymorphonuclear neutrophil

T:

T Lymphocyte

B:

B Lymphocyte

DC:

Dendritic cell

GC:

Giant cell

P:

Plasma cell

Mono:

Monocyte

PR3:

Proteinase 3

LPS:

Lipopolysaccharide

ROS:

Reactive oxygen species

ANCA:

Anti-neutrophil cytoplasmic antibodies

HLE:

Human leukocyte elastase

NO:

Nitric oxide

MPO:

Myeloperoxidase

IL-8:

Interleukin 8

MCP-1:

Monocyte chemoattractant protein-1

TNFα:

Tumor necrosis factor α

IL-1β:

Interleukin 1β

IL-1:

Interleukin 1

ICAM:

Intercellular adhesion molecule

VCAM:

Vascular cell adhesion molecule

PGE2:

Prostaglandin E2

TxB2:

Thromboxane B2

MAC:

Membrane attack complex

TLRs:

Toll-like receptors

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No potential conflicts of interest relevant to this article were reported. Dr. Specks is supported by NIH grant U54 AR057319.

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  1. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Rodrigo Cartin-Ceba, Tobias Peikert & Ulrich Specks

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  1. Rodrigo Cartin-Ceba
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Cartin-Ceba, R., Peikert, T. & Specks, U. Pathogenesis of ANCA-Associated Vasculitis. Curr Rheumatol Rep 14, 481–493 (2012). https://doi.org/10.1007/s11926-012-0286-y

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