ArticleEULAR rheumatology open2025
Long-term risk of malignancies in ANCA-associated vasculitis.
Article in EULAR rheumatology open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Authors and funding
11 authors.
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Abstract
Objectives: Patients with anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV) have an increased malignancy risk, largely attributed to immunosuppressive therapy. Given the latency period for malignancy development, data on long-term incidence in large AAV cohorts remain limited. This study aimed to assess the cumulative malignancy incidence in patients with AAV from European Vasculitis Society (EUVAS) clinical trials (1995-2012). Methods: We analysed 848 patients with AAV from 17 European countries. Data were collected via questionnaires sent to the principal investigators of 7 EUVAS trials. Standardised incidence ratios (SIRs) were calculated using national malignancy databases. Results: At a median follow-up of 8 years (IQR: 2.2-8.8 years), 135 patients experienced 153 malignancies. The overall SIR was 1.40 (95% CI: 1.18-1.64), primarily driven by nonmelanoma skin cancer (NMSC; SIR: 3.52; 95% CI: 2.6-4.7). Excluding NMSC, the SIR was 1.17 (0.96-1.4). The most common malignancies were NMSC (62 cases), prostate (11.1%), and lung (9.8%). The incidence of first malignancy was 2.01 per 100 person-years (95% CI: 1.70-2.39). Cumulative malignancy incidence was 10.8% at 5 years, 19% at 10 years, and 38.2% at 20 years. Patients diagnosed before the age of 40 years had a 30-fold increased risk of NMSC (SIR: 30; 95% CI: 6.03-87.65). Cyclophosphamide use for >6 months, azathioprine duration, and age >65 years were independent risk factors. The diagnosis of malignancy predicted worse survival (log-rank = 9.2; Conclusions: Patients with AAV have a significantly increased risk of NMSC, particularly younger individuals, while solid tumour risk is not significantly elevated. Prolonged cyclophosphamide and azathioprine use were associated with malignancy development.
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