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Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea

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Abstract

The classification system for antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and polyarteritis nodosa had its limitations due to numerous overlapping features of these disease entities. The aim of this study is to investigate the clinical features and outcome of patients diagnosed with microscopic polyangiitis (MPA) according to the newly proposed consensus algorithm of ANCA-associated vasculitides and polyarteritis nodosa. Fifty-five cases of MPA, comprised of 33 men and 22 women, diagnosed according to a new consensus algorithm at a single tertiary hospital were identified for analysis. The main clinical features were constitutional symptoms (78.2%), followed by renal involvement (74.5%), musculoskeletal symptoms (67.3%), skin manifestations (50.9%), neurologic involvement (43.6%), and lung involvement (41.8%). P-ANCA and/or anti-myeloperoxidase antibody were present in 69.1%. Five Factor Score and Birmingham Vasculitis Activity Score (BVAS) at diagnosis were 1.1 ± 0.9 and 10.9 ± 4.9, respectively. Forty-four patients were available for a long-term follow-up, and six patients (13.6%) resulted in death. Mortality was associated with BVAS > 9 at the time of diagnosis, age > 60 years, and presence of cardiomyopathy and interstitial lung disease. The survival rate at 1 and 3 years was 93.9 and 89.2%, respectively. Eight patients (14.5%) required dialysis at the time of diagnosis. This is the first study to demonstrate the clinical features in patients with MPA using a new consensus algorithm. Survival rate was higher than previously reported, and interstitial lung disease was a new risk factor for death in patients with MPA.

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No conflict of interest has been declared by the authors.

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Authors and Affiliations

  1. Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 108 Pyoung-Dong, Jongro-Ku, Seoul, Republic of Korea

    Joong Kyong Ahn

  2. Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 IIwon-Dong, Gangnam-Gu, Seoul, 135-710, Republic of Korea

    Ji-Won Hwang, Jaejoon Lee, Hoon-Suk Cha & Eun-Mi Koh

  3. Department of Internal Medicine, Soonchunhyang University College of Medicine, Bucheon, Gyeonggi-Do, Korea

    Chan Hong Jeon

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  1. Joong Kyong Ahn
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  2. Ji-Won Hwang
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Correspondence to Eun-Mi Koh.

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Joong Kyong Ahn and Ji-Won Hwang equally contributed to this work.

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Ahn, J.K., Hwang, JW., Lee, J. et al. Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in Korea. Rheumatol Int 32, 2979–2986 (2012). https://doi.org/10.1007/s00296-011-2079-4

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  • DOI: https://doi.org/10.1007/s00296-011-2079-4

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