Long-term outcomes remain unchanged despite reduced glucocorticoid exposure in ANCA-associated vasculitis: the...

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 < 0.001 and P = 0.0079, respectively). Over time, GC exposure was significantly reduced in the post-2019 group (P = 0.012), accompanied by fewer infection-related hospitalisations (P < 0.001). However, five-year overall and relapse-free survival did not differ between the pre-2018 and post-2019 groups. Notably, BVAS at 6 and 12 months were significantly higher (P = 0.0070 and P = 0.0057, respectively), and vasculitis-related mortality was more frequent in the post-2019 group (P = 0.0057).

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.