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

作者：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

来源：Clinical and Experimental Nephrology, 2012, 16(4): 580-588.

DOI：10.1007/s10157-012-0598-2

• Anti-neutrophil cytoplasmic auto-antibody (ANCA)
• Immunosuppression
• Prognosis changes
• Rapidly progressive glomerulonephritis (RPGN)
• Vasculitis

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. %26lt;br%26gt;The participants in this retrospective cohort study were 824 ANCA-positive RPGN patients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive. %26lt;br%26gt;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 %26lt; 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). %26lt;br%26gt;Oral prednisolone dose %26lt; 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.

• 出版日期2012-8