start-ver=1.4 cd-journal=joma no-vol=26 cd-vols= no-issue=5 article-no= start-page=730 end-page=737 dt-received= dt-revised= dt-accepted= dt-pub-year=2016 dt-pub=20160311 dt-online= en-article= kn-article= en-subject= kn-subject= en-title= kn-title=Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study en-subtitle= kn-subtitle= en-abstract= kn-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. en-copyright= kn-copyright= en-aut-name=SadaKen-ei en-aut-sei=Sada en-aut-mei=Ken-ei kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=1 ORCID= en-aut-name=HarigaiMasayoshi en-aut-sei=Harigai en-aut-mei=Masayoshi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=2 ORCID= en-aut-name=AmanoKoichi en-aut-sei=Amano en-aut-mei=Koichi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=3 ORCID= en-aut-name=AtsumiTatsuya en-aut-sei=Atsumi en-aut-mei=Tatsuya kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=4 ORCID= en-aut-name=FujimotoShouichi en-aut-sei=Fujimoto en-aut-mei=Shouichi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=5 ORCID= en-aut-name=YuzawaYukio en-aut-sei=Yuzawa en-aut-mei=Yukio kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=6 ORCID= en-aut-name=TakasakiYoshinari en-aut-sei=Takasaki en-aut-mei=Yoshinari kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=7 ORCID= en-aut-name=BannoShogo en-aut-sei=Banno en-aut-mei=Shogo kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=8 ORCID= en-aut-name=SugiharaTakahiko en-aut-sei=Sugihara en-aut-mei=Takahiko kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=9 ORCID= en-aut-name=KobayashiMasaki en-aut-sei=Kobayashi en-aut-mei=Masaki kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=10 ORCID= en-aut-name=UsuiJoichi en-aut-sei=Usui en-aut-mei=Joichi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=11 ORCID= en-aut-name=YamagataKunihiro en-aut-sei=Yamagata en-aut-mei=Kunihiro kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=12 ORCID= en-aut-name=HommaSakae en-aut-sei=Homma en-aut-mei=Sakae kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=13 ORCID= en-aut-name=DobashiHiroaki en-aut-sei=Dobashi en-aut-mei=Hiroaki kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=14 ORCID= en-aut-name=TsuboiNaotake en-aut-sei=Tsuboi en-aut-mei=Naotake kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=15 ORCID= en-aut-name=IshizuAkihiro en-aut-sei=Ishizu en-aut-mei=Akihiro kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=16 ORCID= en-aut-name=SugiyamaHitoshi en-aut-sei=Sugiyama en-aut-mei=Hitoshi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=17 ORCID= en-aut-name=OkadaYasunori en-aut-sei=Okada en-aut-mei=Yasunori kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=18 ORCID= en-aut-name=ArimuraYoshihiro en-aut-sei=Arimura en-aut-mei=Yoshihiro kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=19 ORCID= en-aut-name=MatsuoSeiichi en-aut-sei=Matsuo en-aut-mei=Seiichi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=20 ORCID= en-aut-name=MakinoHirofumi en-aut-sei=Makino en-aut-mei=Hirofumi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=21 ORCID= affil-num=1 en-affil= Department of Nephrology, Rheumatology, Endocrinology and Metabolism , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences kn-affil= affil-num=2 en-affil=Department of Rheumatology, Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University kn-affil= affil-num=3 en-affil=Department of Rheumatology and Clinical Immunology , Saitama Medical Center, Saitama Medical University kn-affil= affil-num=4 en-affil=Division of Rheumatology, Endocrinology and Nephrology at the Graduate School of Medicine , Hokkaido University kn-affil= affil-num=5 en-affil=Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine , Miyazaki University kn-affil= affil-num=6 en-affil=Department of Nephrology , Fujita Health University School of Medicine kn-affil= affil-num=7 en-affil=Department of Internal Medicine and Rheumatology , Juntendo University School of Medicine kn-affil= affil-num=8 en-affil=Division of Rheumatology and Nephrology, Department of Internal Medicine , Aichi Medical University School of Medicine kn-affil= affil-num=9 en-affil=Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology kn-affil= affil-num=10 en-affil=Department of Nephrology , Tokyo Medical University Ibaraki Medical Center kn-affil= affil-num=11 en-affil=Department of Nephrology, Faculty of Medicine , University of Tsukuba kn-affil= affil-num=12 en-affil=Department of Nephrology, Faculty of Medicine , University of Tsukuba kn-affil= affil-num=13 en-affil=Department of Respiratory Medicine , Toho University Omori Medical Center kn-affil= affil-num=14 en-affil=Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University kn-affil= affil-num=15 en-affil=Department of Nephrology, Internal Medicine , Nagoya University Graduate School of Medicine kn-affil= affil-num=16 en-affil=Faculty of Health Sciences , Hokkaido University kn-affil= affil-num=17 en-affil=Department of Chronic Kidney Disease and Peritoneal Dialysis , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences kn-affil= affil-num=18 en-affil=Department of Pathology , Keio University School of Medicine kn-affil= affil-num=19 en-affil=Nephrology and Rheumatology, First Department of Internal Medicine , Kyorin University School of Medicine kn-affil= affil-num=20 en-affil=Department of Nephrology, Internal Medicine , Nagoya University Graduate School of Medicine kn-affil= affil-num=21 en-affil=Okayama University Hospital kn-affil= en-keyword=Antineutrophil cytoplasmic antibody-associated vasculitis kn-keyword=Antineutrophil cytoplasmic antibody-associated vasculitis en-keyword=Eosinophilic granulomatosis with polyangiitis kn-keyword=Eosinophilic granulomatosis with polyangiitis en-keyword=Granulomatosis with polyangiitis kn-keyword=Granulomatosis with polyangiitis en-keyword=Inception cohort kn-keyword=Inception cohort en-keyword=Microscopic polyangiitis kn-keyword=Microscopic polyangiitis END