Ken-Ei Sada1, Masayoshi Harigai2,3, Koichi Amano4, Tatsuya Atsumi5, Shouichi Fujimoto6, Yukio Yuzawa7, Yoshinari Takasaki8, Shogo Banno9, Takahiko Sugihara10, Masaki Kobayashi11, Joichi Usui12, Kunihiro Yamagata12, Sakae Homma13, Hiroaki Dobashi14, Naotake Tsuboi15, Akihiro Ishizu16, Hitoshi Sugiyama17, Yasunori Okada18, Yoshihiro Arimura19, Seiichi Matsuo15, Hirofumi Makino20. 1. a Department of Nephrology, Rheumatology, Endocrinology and Metabolism , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan . 2. b Department of Pharmacovigilance . 3. c Department of Rheumatology, Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University , Tokyo , Japan . 4. d Department of Rheumatology and Clinical Immunology , Saitama Medical Center, Saitama Medical University , Saitama , Japan . 5. e Division of Rheumatology, Endocrinology and Nephrology at the Graduate School of Medicine , Hokkaido University , Hokkaido , Japan . 6. f Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine , Miyazaki University , Miyazaki , Japan . 7. g Department of Nephrology , Fujita Health University School of Medicine , Aichi , Japan . 8. h Department of Internal Medicine and Rheumatology , Juntendo University School of Medicine, Tokyo , Japan . 9. i Division of Rheumatology and Nephrology, Department of Internal Medicine , Aichi Medical University School of Medicine , Aichi , Japan . 10. j Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology , Tokyo , Japan . 11. k Department of Nephrology , Tokyo Medical University Ibaraki Medical Center , Ibaraki , Japan . 12. l Department of Nephrology, Faculty of Medicine , University of Tsukuba , Ibaraki , Japan . 13. m Department of Respiratory Medicine , Toho University Omori Medical Center , Tokyo , Japan . 14. n Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine , Kagawa University , Kagawa , Japan . 15. o Department of Nephrology, Internal Medicine , Nagoya University Graduate School of Medicine , Aichi , Japan . 16. p Faculty of Health Sciences , Hokkaido University , Hokkaido , Japan . 17. q Department of Chronic Kidney Disease and Peritoneal Dialysis , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan . 18. r Department of Pathology , Keio University School of Medicine , Tokyo , Japan . 19. s Nephrology and Rheumatology, First Department of Internal Medicine , Kyorin University School of Medicine , Tokyo , Japan , and. 20. t Okayama University Hospital , Okayama , Japan.
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.
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.
Entities:
Keywords:
Antineutrophil cytoplasmic antibody-associated vasculitis; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Inception cohort; Microscopic polyangiitis