Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. Issue 5 (2nd September 2016)

• Record Type: Journal Article
• Authors: Sada, Ken-ei; Harigai, Masayoshi; Amano, Koichi; Atsumi, Tatsuya; Fujimoto, Shouichi; Yuzawa, Yukio; Takasaki, Yoshinari; Banno, Shogo; Sugihara, Takahiko; Kobayashi, Masaki; Usui, Joichi; Yamagata, Kunihiro; Homma, Sakae; Dobashi, Hiroaki; Tsuboi, Naotake; Ishizu, Akihiro; Sugiyama, Hitoshi; Okada, Yasunori; Arimura, Yoshihiro; Matsuo, Seiichi; Makino, Hirofumi
• Is Part Of: Modern rheumatology. Volume 26:Issue 5(2016)
• Page Start: 730
• Page End: 737
• Publication Date: 2016-09-02
• Subjects: Antineutrophil cytoplasmic antibody-associated vasculitis -- Eosinophilic granulomatosis with polyangiitis -- Granulomatosis with polyangiitis -- Inception cohort -- Microscopic polyangiitis; Rheumatology -- Periodicals; 616.723005
• DOI: 10.3109/14397595.2016.1140274
• Languages: English
• ISSNs: 1439-7595

Abstract

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.