ANCA-associated systemic vasculitis in Japan: clinical features and prognostic changes.

Yamagata, Kunihiro; Usui, Joichi; Saito, Chie; Yamaguchi, Naoto; Hirayama, Kouichi; Mase, Kaori; Kobayashi, Masaki; Koyama, Akio; Sugiyama, Hitoshi; Nitta, Kosaku; Wada, Takashi; Muso, Eri; Arimura, Yoshihiro; Makino, Hirofumi; Matsuo, Seiichi

Background: This study was conducted to standardize treatment and determine patient and renal outcome in Japanese anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) patients, because the prognosis of AAV/RPGN patients in Japan had been poor compared with that of other countries. Methods: The participants in this retrospective cohort study were 824 ANCA-positive RPGN patients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive. Results: Among the early-years cohort (group A; cases diagnosed between 1988 and 1998), patients frequently died due to opportunistic infection. Therefore, we recommended a reduced dose of prednisolone (oral prednisolone dose <0.8 mg/kg/day) with or without cyclophosphamide for initial treatment of Japanese RPGN patients. After this recommendation, 1-year survival of the patients improved: 75% in group A, 79% in group B (between 1999 and 2002), and 81% in group C (after 2003). During the entire observation period, average serum creatinine level at the start of treatment decreased, and improvement of 1-year renal survival was also found (72% in group A, 83% in group B, and 83% in group C), while the recurrence rate was significantly increased in group C (0.05/patient-year in group A, 0.07/patient-year in group B, and 0.13/patient-year in group C). Conclusions: Oral prednisolone dose <0.8 mg/kg/day with or without cyclophosphamide as an initial treatment could improve patient survival in older Japanese AAV/RPGN patients. However, maintenance treatment avoiding relapse should be established to improve renal outcomes.

JAPAN; VASCULITIS; GLOMERULONEPHRITIS; MYELOPEROXIDASE; PREDNISOLONE; CYCLOPHOSPHAMIDE; HEALTH outcome assessment; IMMUNOSUPPRESSION

Clinical & Experimental Nephrology, 2012, Vol 16, Issue 4, p580

1342-1751

Academic Journal

10.1007/s10157-012-0598-2