title: Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study.
abstract: BACKGROUND
  This study aims to elucidate the prognosis and the effectiveness of current treatments for Japanese patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).
  
  
  METHODS
  Patients with newly diagnosed MPA and GPA were enrolled in a nationwide, prospective, inception cohort study from 22 tertiary Japanese institutions, and treatment patterns and responses were evaluated for 24 months. Primary outcome measures were rates of remission (Birmingham Vasculitis Activity Score, 0) and remission with low-dose glucocorticoids (GC) (prednisolone ≤ 10 mg) (GC remission).
  
  
  RESULTS
  Of 156 enrolled patients, 78 MPA patients and 33 GPA patients were included. Concomitant cyclophosphamide (CY) was used in 24 MPA (31 %) and 20 GPA (60 %) patients during the initial 3 weeks of treatment. After 6 months, remission was achieved in 66 MPA (85 %) and 29 GPA (87 %) patients, while GC remission was obtained in only 31 MPA (40 %) and 13 GPA (39 %) patients. During the 24-month period, 14 MPA patients and 2 GPA patients died; end stage renal disease (ESRD) was noted in 13 MPA patients but no GPA patients. Patients with severe disease, according to the European Vasculitis Study Group (EUVAS) classification, showed poorer ESRD-free and overall survival rates than those with generalized disease (p < 0.0001). There were no differences in relapse-free survival rates between GPA and MPA, among EUVAS-defined disease severity categories, and between anti-neutrophil cytoplasmic antibody subspecialties.
  
  
  CONCLUSIONS
  The majority of Japanese patients with MPA and GPA received treatment with high-dose GC and limited CY use, and showed high remission and relapse-free survival rates but low GC remission rates in clinical practice.
  
  
  BACKGROUND
  University Hospital Medical Information Network Clinical Trials Registry UMIN000001648 . Registered 28 February 2009.
authors.affiliation: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan. sadakenn@md.okayama-u.ac.jp.
authors.forename: Ken-ei
authors.initials: KE
authors.lastname: Sada
authors.affiliation: Center for Rheumatology, Okayama Saiseikai General Hospital, 1-17-18 Ifuku-cho, Kita-ku, Okayama, 700-8511, Japan. yamamura@saiseidr.jp.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yamamura
authors.affiliation: Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. harigai.masayoshi@tmdtwmu.ac.jp.|Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. harigai.masayoshi@tmdtwmu.ac.jp.
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Harigai
authors.affiliation: Department of the Control for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan. takfujii@kuhp.kyoto-u.ac.jp.
authors.forename: Takao
authors.initials: T
authors.lastname: Fujii
authors.affiliation: Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan. tyoshi@juntendo.ac.jp.
authors.forename: Yoshinari
authors.initials: Y
authors.lastname: Takasaki
authors.affiliation: Department of Rheumatology and Clinical Immunology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Saitama, 350-8550, Japan. amanokoi@saitama-med.ac.jp.
authors.forename: Koichi
authors.initials: K
authors.lastname: Amano
authors.affiliation: Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, 5200 Kiyotakecho Kihara, Miyazaki, 889-1692, Japan. fujimos@med.miyazaki-u.ac.jp.
authors.forename: Shouichi
authors.initials: S
authors.lastname: Fujimoto
authors.affiliation: Center for Nephrology and Urology, Division of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 2-4-20 Ohgimachi, Osaka, 530-8480, Japan. muso@kitano-hp.or.jp.
authors.forename: Eri
authors.initials: E
authors.lastname: Muso
authors.affiliation: Department of Rheumatology, Shimane University Faculty of Medicine, 89-1 Enya-cho, Izumo, 693-8501, Japan. murakawa@med.shimane-u.ac.jp.
authors.forename: Yohko
authors.initials: Y
authors.lastname: Murakawa
authors.affiliation: Nephrology and Rheumatology, First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan. arimuray@ks.kyorin-u.ac.jp.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.affiliation: Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan. makino@md.okayama-u.ac.jp.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D018572:Disease-Free Survival; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000145:classification / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D007676:Kidney Failure, Chronic / Q000175:diagnosis; D008297:Male; D055953:Microscopic Polyangiitis / Q000145:classification / Q000188:drug therapy* / Q000473:pathology; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use*; D011446:Prospective Studies; D012008:Recurrence; D012074:Remission Induction; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine