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


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13

 

  1. Medical school of Chinese PLA, Beijing; and Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  2. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing; and State Key Laboratory of Kidney Diseases, Chinese PLA General Hospital, Beijing, China.
  3. Department of Rheumatology and Immunology, the Fourth Medical Center, Chinese PLA General Hospital, Beijing, China.
  4. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  5. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  6. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  7. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  8. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  9. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing, China.
  10. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing 100853, China
  11. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing 100853, China
  12. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing; and State Key Laboratory of Kidney Diseases, Chinese PLA General Hospital, Beijing, China.
  13. Department of Rheumatology and Immunology, the First Medical Center, Chinese PLA General Hospital, Beijing; and State Key Laboratory of Kidney Diseases, Chinese PLA General Hospital, Beijing, China. jian_jzhu@126.com

CER18452
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PMID: 40242911 [PubMed]

Received: 17/12/2024
Accepted : 24/02/2025
In Press: 09/04/2025

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.

DOI: https://doi.org/10.55563/clinexprheumatol/5c7nti

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