Yonsei Med J.  2019 Jan;60(1):10-21. 10.3349/ymj.2019.60.1.10.

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Korea: A Narrative Review

• Choi CB
• Park YB
• Lee SW

• KMID: 2428273
• DOI: http://doi.org/10.3349/ymj.2019.60.1.10

Abstract

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeable advancements in studies on the pathophysiology of AAV and the classification criteria for AAV in Western countries. However, studies analysing clinical features of Korean patients with AAV have only been conducted and reported since 2000. One year-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitis activity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortality or poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infection free survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed 1.43 compared to the general Korean population.

Keyword

Antineutrophil cytoplasmic antibody; vasculitis; Korea

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Classification
Consensus
Cytoplasm*
Eosinophils
Granulomatosis with Polyangiitis
Humans
Incidence
Korea*
Microscopic Polyangiitis
Mortality
Rheumatology
Survival Rate
Vasculitis*
Antibodies, Antineutrophil Cytoplasmic

Figure

• 

  Fig. 1 Mechanism of ANCA-associated vasculitis. (A) Priming and activation of neutrophils, (B) expression of adhesion molecule on endothelial cells, (C) binding of pathogenic ANCA to ANCA-antigens, (D) interaction between neutrophils and endothelial adhesion molecules, leading to extravasation of neutrophils, (E) production of reactive oxygen radicals and degranulation of neutrophils, (F) a loop of complement activating factors to C5a fragment. N, neutrophil; Ed, endothelium; CD, cluster of differentiation; VCAM-1, vascular cell adhesion molecule 1; ICAM-1, intercellular adhesion molecule 1; ANCA, antineutrophil cytoplasm antibody; O, oxygen; C, complement; BbP, factor B properdin.

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