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

박용범|이상원|최찬범
연세대학교의과대학 (Yonsei University College of Medicine)|2019.01|Yonsei Medical Journal|제60권 제1호|pp. 10-21|DOI 10.3349/ymj.2019.60.1.10

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키워드

KoreaAntineutrophil cytoplasmic antibodyvasculitis
주제분류의학일반

초록

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involvesmall vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations andpathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), andeosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currentlythe algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclatureof Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeableadvancements 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. Oneyear-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitisactivity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortalityor poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infectionfree survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed1.43 compared to the general Korean population.

Abstract

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involvesmall vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations andpathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), andeosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currentlythe algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclatureof Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeableadvancements 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. Oneyear-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitisactivity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortalityor poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infectionfree survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed1.43 compared to the general Korean population.

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