Objective: To evaluate standardized incidence ratios (SIRs) of cancer in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Methods: A systematic review and meta-analysis of studies from Medline, Embase, and Cochrane databases (inception to May 2025) was performed. Pooled SIRs were analyzed overall and by region, sex, age, AAV subtype, and cancer type.
Results: Fourteen studies involving 5553 AAV patients and 883 cancers were included. Overall cancer risk was significantly elevated (SIR: 1.767, 95% confidence interval [CI] 1.514-2.063). Regionally, SIRs were increased in Europe (1.717), Asia (2.193), and Oceania (1.740; all p < 0.001). Age-specific analyses showed elevated risks in patients ≤ 60 years (SIR: 2.217) and > 60 years (SIR: 2.072). Notably increased risks were observed for bladder cancer (SIR: 4.291), leukemia (4.082), kidney cancer (3.202), nonmelanoma skin cancer (3.598), lung cancer (2.082), malignant melanoma (1.701), liver cancer (1.778), oral cavity (1.760), brain (1.912), and colorectal cancer (1.465). No significant increase was found for breast, prostate, stomach cancer, non-Hodgkin lymphoma (NHL), or lymphoma.
Conclusion: Patients with AAV face a significantly increased risk of cancer, particularly bladder, hematologic, kidney, and skin cancers. These findings support the need for tailored cancer surveillance strategies in AAV management.
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