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

Area of Science:

• Nephrology
• Immunology
• Rheumatology

Background:

• Japanese patients with ANCA-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) historically had poor prognoses.
• Standardizing treatment and evaluating outcomes were necessary to improve patient and renal survival.

Purpose of the Study:

• To standardize treatment protocols for Japanese AAV/RPGN patients.
• To determine patient and renal outcomes in this population.
• To compare outcomes with international data.

Main Methods:

• Retrospective cohort study of 824 ANCA-positive RPGN patients.
• Analysis of patient and renal outcomes based on treatment periods (1988-1998, 1999-2002, post-2003).
• Focus on myeloperoxidase (MPO)-ANCA positive patients (705/824).

Main Results:

• Reduced initial prednisolone dosage (<0.8 mg/kg/day) with or without cyclophosphamide improved 1-year patient survival rates.
• Serum creatinine levels decreased, and 1-year renal survival improved post-recommendation.
• However, the rate of AAV/RPGN recurrence significantly increased in the later treatment group.

Conclusions:

• Lower initial oral prednisolone doses (<0.8 mg/kg/day) with or without cyclophosphamide improve survival in older Japanese AAV/RPGN patients.
• Effective maintenance treatment strategies are crucial to prevent relapses and improve long-term renal outcomes.