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The co-occurrence of IgG4-related disease and malignancy: insights from a prospective cohort study

Clin Exp Rheumatol. 2025 Apr 9. doi: 10.55563/clinexprheumatol/5c7nti. Online ahead of print.

ABSTRACT

OBJECTIVES: This study investigates the relationship between IgG4-related disease (IgG4-RD) and malignancies, focusing on tumour distribution and risk factors for malignancy development during follow-up.

METHODS: We analysed a prospective cohort of 278 IgG4-RD patients, including 22 with malignancies, and calculated standardised incidence ratios (SIRs). Bayesian statistical models were employed to identify risk factors.

RESULTS: Among 278 IgG4-RD patients, 22 (7.9%) developed malignancies. Lung cancer (13.6%) was the most prevalent malignancy, with a significantly higher incidence in the follow-up group compared to the history/concurrent group (19.3% vs. 7.0%; p=0.008). Testicular cancer occurred exclusively in the history/concurrent group (3.5%) and was absent in the follow-up group (p=0.044). The overall SIR was 3.28 (95% CI: 1.31-5.58) and lymphoma exhibited a markedly high SIR of 17.63 (95% CI: 0-52.89). Elevated ESR (estimate: 0.12, 95% CI: 0.04-0.23) and more organ involvements (estimate: 1.94, 95% CI: 0.31-3.93) were identified as significant risk factors for malignancy development.

CONCLUSIONS: IgG4-RD patients exhibit an elevated risk of specific malignancies, particularly lymphoma, with elevated ESR and greater organ involvement identified as significant risk factors for malignancy development during follow-up period.

PMID:40242911 | DOI:10.55563/clinexprheumatol/5c7nti

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