Current Rheumatology Reports

, Volume 14, Issue 6, pp 481–493

Pathogenesis of ANCA-Associated Vasculitis

  • Rodrigo Cartin-Ceba
  • Tobias Peikert
  • Ulrich Specks
VASCULITIS (LR ESPINOZA, SECTION EDITOR)

DOI: 10.1007/s11926-012-0286-y

Cite this article as:
Cartin-Ceba, R., Peikert, T. & Specks, U. Curr Rheumatol Rep (2012) 14: 481. doi:10.1007/s11926-012-0286-y

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.

Keywords

ANCAANCA-associated vasculitisGranulomatosis with polyangiitisMicroscopic polyangiitisEosinophilic granulomatosis with polyangiitisPathogenesisEtiology

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

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Rodrigo Cartin-Ceba
    • 1
  • Tobias Peikert
    • 1
  • Ulrich Specks
    • 1
  1. 1.Division of Pulmonary and Critical Care MedicineMayo ClinicRochesterUSA