title: Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.
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.
authors.affiliation: a Department of Nephrology, Rheumatology, Endocrinology and Metabolism , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan .
authors.forename: Ken-Ei
authors.initials: KE
authors.lastname: Sada
authors.affiliation: b Department of Pharmacovigilance .|c Department of Rheumatology, Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University , Tokyo , Japan .
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Harigai
authors.affiliation: d Department of Rheumatology and Clinical Immunology , Saitama Medical Center, Saitama Medical University , Saitama , Japan .
authors.forename: Koichi
authors.initials: K
authors.lastname: Amano
authors.affiliation: e Division of Rheumatology, Endocrinology and Nephrology at the Graduate School of Medicine , Hokkaido University , Hokkaido , Japan .
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Atsumi
authors.affiliation: f Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine , Miyazaki University , Miyazaki , Japan .
authors.forename: Shouichi
authors.initials: S
authors.lastname: Fujimoto
authors.affiliation: g Department of Nephrology , Fujita Health University School of Medicine , Aichi , Japan .
authors.forename: Yukio
authors.initials: Y
authors.lastname: Yuzawa
authors.affiliation: h Department of Internal Medicine and Rheumatology , Juntendo University School of Medicine, Tokyo , Japan .
authors.forename: Yoshinari
authors.initials: Y
authors.lastname: Takasaki
authors.affiliation: i Division of Rheumatology and Nephrology, Department of Internal Medicine , Aichi Medical University School of Medicine , Aichi , Japan .
authors.forename: Shogo
authors.initials: S
authors.lastname: Banno
authors.affiliation: j Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology , Tokyo , Japan .
authors.forename: Takahiko
authors.initials: T
authors.lastname: Sugihara
authors.affiliation: k Department of Nephrology , Tokyo Medical University Ibaraki Medical Center , Ibaraki , Japan .
authors.forename: Masaki
authors.initials: M
authors.lastname: Kobayashi
authors.affiliation: l Department of Nephrology, Faculty of Medicine , University of Tsukuba , Ibaraki , Japan .
authors.forename: Joichi
authors.initials: J
authors.lastname: Usui
authors.affiliation: l Department of Nephrology, Faculty of Medicine , University of Tsukuba , Ibaraki , Japan .
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yamagata
authors.affiliation: m Department of Respiratory Medicine , Toho University Omori Medical Center , Tokyo , Japan .
authors.forename: Sakae
authors.initials: S
authors.lastname: Homma
authors.affiliation: n Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University , Kagawa , Japan .
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Dobashi
authors.affiliation: o Department of Nephrology, Internal Medicine , Nagoya University Graduate School of Medicine , Aichi , Japan .
authors.forename: Naotake
authors.initials: N
authors.lastname: Tsuboi
authors.affiliation: p Faculty of Health Sciences , Hokkaido University , Hokkaido , Japan .
authors.forename: Akihiro
authors.initials: A
authors.lastname: Ishizu
authors.affiliation: q Department of Chronic Kidney Disease and Peritoneal Dialysis , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan .
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sugiyama
authors.affiliation: r Department of Pathology , Keio University School of Medicine , Tokyo , Japan .
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Okada
authors.affiliation: s Nephrology and Rheumatology, First Department of Internal Medicine , Kyorin University School of Medicine , Tokyo , Japan , and.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.affiliation: o Department of Nephrology, Internal Medicine , Nagoya University Graduate School of Medicine , Aichi , Japan .
authors.forename: Seiichi
authors.initials: S
authors.lastname: Matsuo
authors.affiliation: t Okayama University Hospital , Okayama , Japan.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
mesh_terms: D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000276:immunology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Antineutrophil cytoplasmic antibody-associated vasculitis; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Inception cohort; Microscopic polyangiitis