Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

Ken Ei Sada, Masayoshi Harigai, Koichi Amano, Tatsuya Atsumi, Shouichi Fujimoto, Yukio Yuzawa, Yoshinari Takasaki, Shogo Banno, Takahiko Sugihara, Masaki Kobayashi, Joichi Usui, Kunihiro Yamagata, Sakae Homma, Hiroaki Dobashi, Naotake Tsuboi, Akihiro Ishizu, Hitoshi Sugiyama, Yasunori Okada, Yoshihiro Arimura, Seiichi MatsuoHirofumi Makino

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Objective: To compare disease severity classification systems for six-month outcome prediction in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods: Patients with newly diagnosed AAV from 53 tertiary institutions were enrolled. Six-month remission, overall survival, and end-stage renal disease (ESRD)-free survival were evaluated. Results: According to the European Vasculitis Study Group (EUVAS)-defined disease severity, the 321 enrolled patients were classified as follows: 14, localized; 71, early systemic; 170, generalized; and 66, severe disease. According to the rapidly progressive glomerulonephritis (RPGN) clinical grading system, the patients were divided as follows: 60, grade I; 178, grade II; 66, grade III; and 12, grade IV. According to the Five-Factor Score (FFS) 2009, 103, 109, and 109 patients had ≤1, 2, and ≥3 points, respectively. No significant difference in remission rates was found in any severity classification. The overall and ESRD-free survival rates significantly differed between grades I/II, III, and IV, regardless of renal involvement. Severe disease was a good predictor of six-month overall and ESRD-free survival. The FFS 2009 was useful to predict six-month ESRD-free survival but not overall survival. Conclusions: The RPGN grading system was more useful to predict six-month overall and ESRD-free survival than the EUVAS-defined severity or FFS 2009.

Original languageEnglish
Pages (from-to)730-737
Number of pages8
JournalModern Rheumatology
Volume26
Issue number5
DOIs
Publication statusPublished - Sep 2 2016

Keywords

  • Antineutrophil cytoplasmic antibody-associated vasculitis
  • Eosinophilic granulomatosis with polyangiitis
  • Granulomatosis with polyangiitis
  • Inception cohort
  • Microscopic polyangiitis

ASJC Scopus subject areas

  • Rheumatology

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    Sada, K. E., Harigai, M., Amano, K., Atsumi, T., Fujimoto, S., Yuzawa, Y., Takasaki, Y., Banno, S., Sugihara, T., Kobayashi, M., Usui, J., Yamagata, K., Homma, S., Dobashi, H., Tsuboi, N., Ishizu, A., Sugiyama, H., Okada, Y., Arimura, Y., ... Makino, H. (2016). Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. Modern Rheumatology, 26(5), 730-737. https://doi.org/10.3109/14397595.2016.1140274