Meta-Analysis of Mortality Patterns in ANCA-Associated Vasculitis Across Regions, Sex, and Subtypes

초록

Objectives To evaluate all-cause and cause-specific standardized mortality ratios (SMRs) in patients with ANCA-associated vasculitis (AAV) using meta-analyses stratified by sex, geographic region, and disease subtype.Methods A comprehensive systematic review was conducted using the Medline, Embase, and Web of Sciences databases from inception to April 2025. Meta-analyses were performed to assess all-cause, sex-specific, region-specific, disease-specific, and cause-specific SMRs.Results Twenty-two studies comprising 8,749 AAV patients and 2,503 deaths were included in the meta-analysis. Overall all-cause SMR was significantly elevated in AAV patients compared with the general population (SMR: 2.477, 95% CI: 2.028-3.026, p<0.001). Region-specific analyses showed significantly increased SMRs in Europe (SMR: 2.447, 95% CI: 2.000-2.893), North America (SMR: 2.782, 95% CI: 1.086-4.478), and Asia (SMR: 2.364, 95% CI: 1.057-3.672), with the highest SMR observed in Oceania (SMR: 3.300, 95% CI: 2.900-3.755). Sex-specific analysis revealed significantly elevated SMRs in both women (SMR: 2.749, 95% CI: 1.613-4.684, p<0.001) and men (SMR: 2.481, 95% CI: 1.716-3.587, p<0.001). Among AAV subtypes, patients having granulomatosis with polyangiitis had the highest SMR (SMR: 2.686, 95% CI: 2.001-3.605), followed by patients with microscopic polyangiitis (SMR: 2.508, 95% CI: 1.798-3.500) and those having eosinophilic granulomatosis with polyangiitis (SMR: 1.924, 95% CI: 1.338-2.765); all differences were statistically significant (p<0.001). SMR was significantly higher for MPO-ANCA (2.548, 95% CI: 2.089-3.107, p<0.001) than for PR3-ANCA (1.769, 95% CI: 1.188-2.634, p=0.025). Cause-specific analyses revealed significantly increased SMRs in patients with malignancy (SMR: 2.319, 95% CI: 1.871-2.875, p<0.001), renal disease (SMR: 2.478, 95% CI: 1.579-3.962, p<0.001), respiratory disease (SMR: 2.000, 95% CI: 0.926-4.320, p<0.001), cardiovascular disease (CVD) (SMR: 2.300, 95% CI: 1.485-3.563, p<0.001), and infection (SMR: 13.964, 95% CI: 7.893-24.48, p<0.001), with those having infection showing the most pronounced increase in mortality risk.Conclusion This meta-analysis demonstrates a significantly increased risk of mortality among AAV patients, especially those with infections, malignancy, renal complications, and cardiovascular causes. Regional, sex-based, and subtype differences further underscore the importance of tailored monitoring and management strategies in this population.

키워드