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| Item Type: | Article |
|---|---|
| Title: | Long-term risk of malignancies in ANCA-associated vasculitis |
| Creators: |
Sanchez Alamo, Beatriz, Gonzalez Chiappe, Solange  ORCID: https://orcid.org/0000-0001-9013-5088, Moi, Laura ORCID: https://orcid.org/0000-0001-7890-944X, Bajema, Ingeborg, Faurschou, Mikkel, Flossmann, Oliver, Heijl, Caroline, Jayne, David, Mahr, Alfred and Westman, Kerstin ORCID: https://orcid.org/0000-0002-2930-998X
|
| 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; = .002). 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. |
| Source: | EULAR Rheumatology Open |
| ISSN: | 3050-7081 |
| Publisher: | Elsevier / European Alliance of Associations for Rheumatology (EULAR) |
| Volume: | 1 |
| Number: | 3 |
| Page Range: | 272-281 |
| Date: | October 2025 |
| Official Publication: | https://doi.org/10.1016/j.ero.2025.08.007 |
| PubMed: | View item in PubMed |
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