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

• Published in: Arthritis research & therapy 2015-11, Vol.17 (305), p.305-305, Article 305
• Main Authors: Sada, Ken-ei, Yamamura, Masahiro, Harigai, Masayoshi, Fujii, Takao, Takasaki, Yoshinari, Amano, Koichi, Fujimoto, Shouichi, Muso, Eri, Murakawa, Yohko, Arimura, Yoshihiro, Makino, Hirofumi
• Format: Article
• Language: English
• Subjects: Aged; Aged, 80 and over; Arthritis; Azathioprine - therapeutic use; Care and treatment; Chronic kidney failure; Complications and side effects; Corticosteroids; Cyclophosphamide - therapeutic use; Diagnosis; Disease-Free Survival; Drug Therapy, Combination; Female; Glucocorticoids - therapeutic use; Granulomatosis with Polyangiitis - classification; Granulomatosis with Polyangiitis - drug therapy; Granulomatosis with Polyangiitis - pathology; Humans; Immunosuppressive Agents - therapeutic use; Japan; Kidney Failure, Chronic - diagnosis; Male; Microscopic Polyangiitis - classification; Microscopic Polyangiitis - drug therapy; Microscopic Polyangiitis - pathology; Middle Aged; Prednisolone; Prednisolone - therapeutic use; Prospective Studies; Recurrence; Remission Induction; Severity of Illness Index; Treatment Outcome
• ISSN: 1478-6354; 1478-6362; 1478-6354
• DOI: 10.1186/s13075-015-0815-y

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). 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). 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