Japanese AAV Cohort Shows MPA Has 67.6% Five-Year Survival; Glucocorticoid Reduction Not Linked to Better Outcomes

The long-term prognosis of patients with anti-neutrophil cytoplasmic antibody–associated vasculitis (AAV) has historically been poor. In recent years, the widespread use of novel targeted therapies has led to an increased emphasis on reduced-dose glucocorticoid (GC) strategies; however, owing to regional differences in the frequency and clinical characteristics of AAV subtypes, contemporary real-world evidence on long-term outcomes in Japanese patient populations remains limited. The primary outcome was to elucidate subtype-specific clinical characteristics and five-year overall and relapse-free survival in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Secondary outcomes included temporal changes in treatment practices and prognosis, stratified by year of diagnosis. We conducted a multicentre retrospective study using data from the REVEAL cohort. A total of 460 newly diagnosed, treatment-naïve cases were included (microscopic polyangiitis (MPA), n = 283; granulomatosis with polyangiitis (GPA), n = 66; eosinophilic granulomatosis with polyangiitis (EGPA), n = 111). Clinical characteristics, five-year relapse-free and overall survival were evaluated. To assess temporal changes in treatment practices and prognosis, patients were stratified by median year of diagnosis (2018) into pre-2018 and post-2019 groups. MPA showed the poorest prognosis among AAV subtypes, with a five-year overall survival rate of 67.6%. Older age and impaired renal function were independently associated with increased mortality (P  MPA remained the AAV subtype with the poorest prognosis. Although GC exposure has decreased in recent years, this trend was not associated with improved clinical outcomes in the present cohort.