J Korean Rheum Assoc.  2010 Jun;17(2):108-132. 10.4078/jkra.2010.17.2.108.

ANCA Associated Vasculitis

• Uhm WS

• KMID: 2201951
• DOI: http://doi.org/10.4078/jkra.2010.17.2.108

Abstract

Primary antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) constitute a group of small vessel vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. Recently, many in vitro and in vivo studies have highlighted the role of ANCA as the main pathophysiological factor in the development of AAV. Two remarkable studies on ANCA pathogenesis were recently reported. One study examined anti-lysosomal membrane protein-2, which supports the 'shared epitope' theory. The other examined the neutrophil extracellular trap that is released by neutrophils primed by ANCA. Each disease of AAV shows a broad spectrum of the clinical features and severities, which makes it difficult to diagnose and treat them. Considerable effort has been made in the past decades to improve the treatment outcomes, reduce the incidence of relapse and avoid drug toxicity. This review describes the current understanding of AAV along with a few Korean reports.

Keyword

ANCA associated vasculitis; Small vessel vasculitis; Wegener's granulomatosis; Microscopic polyangiitis; Churg-Strauss syndrome

MeSH Terms

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Churg-Strauss Syndrome
Drug Toxicity
Glycosaminoglycans
Incidence
Membranes
Microscopic Polyangiitis
Neutrophils
Recurrence
Vasculitis
Wegener Granulomatosis
Antibodies, Antineutrophil Cytoplasmic
Glycosaminoglycans

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