Annals of the rheumatic diseases · 2026
IgG4-related disease is not associated with an increased incidence of malignancy in a large, single-centre cohort.
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Michelson Daniel A, McMahon Grace A, Jha Isha, Jiang Bohang, Zhang Yuqing, Fernandes Ana D, Stone John H, Wallace Zachary S, Katz Guy
Аннотация
OBJECTIVES: Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated disease-causing inflammation and fibrosis in multiple organs, often associated with elevated serum IgG4 concentrations. Whether IgG4-RD is associated with malignancy remains unclear. We investigated whether there is an association between IgG4-RD and incident malignancy.
METHODS: We performed a single-centre, retrospective cohort study of patients who fulfilled the American College of Rheumatology/European Alliance of Associations for Rheumatology IgG4-RD Classification Criteria. Malignancy history was collected by survey and medical record review. Age- and sex-stratified malignancy incidence rates in the cohort were compared with rates in the general US population. Cox proportional hazards were used to identify disease characteristics associated with incident malignancy.
RESULTS: Of the 348 patients with IgG4-RD in our cohort, 34 (10%) developed incident malignancies over a median follow-up of 8.04 years (IQR, 1.35-14.72 years). The most common malignancies were lymphoma (n = 6, 16% of malignancies), prostate cancer (n = 6, 16%), and lung cancer (n = 5, 13%). Compared with the general US population, there was no increased incidence of malignancy in patients with IgG4-RD (standardised incidence ratio, 0.88 [0.61-1.23]). Among patients with IgG4-RD, those who ever smoked (adjusted hazard ratio [HR], 2.46 [1.16-5.24]) or were older (HR, 1.35 [1.00-1.83] per decade) had increased risk of malignancy, whereas female patients (HR, 0.37 [0.14-0.96]) and those with submandibular gland involvement (HR, 0.25 [0.10-0.61]) had decreased risk.
CONCLUSIONS: IgG4-RD was not associated with an increased incidence of malignancy following IgG4-RD onset. Among patients with IgG4-RD, smoking and older age at IgG4-RD onset were associated with higher malignancy risk. Female sex and submandibular gland involvement were associated with lower malignancy risk.
METHODS: We performed a single-centre, retrospective cohort study of patients who fulfilled the American College of Rheumatology/European Alliance of Associations for Rheumatology IgG4-RD Classification Criteria. Malignancy history was collected by survey and medical record review. Age- and sex-stratified malignancy incidence rates in the cohort were compared with rates in the general US population. Cox proportional hazards were used to identify disease characteristics associated with incident malignancy.
RESULTS: Of the 348 patients with IgG4-RD in our cohort, 34 (10%) developed incident malignancies over a median follow-up of 8.04 years (IQR, 1.35-14.72 years). The most common malignancies were lymphoma (n = 6, 16% of malignancies), prostate cancer (n = 6, 16%), and lung cancer (n = 5, 13%). Compared with the general US population, there was no increased incidence of malignancy in patients with IgG4-RD (standardised incidence ratio, 0.88 [0.61-1.23]). Among patients with IgG4-RD, those who ever smoked (adjusted hazard ratio [HR], 2.46 [1.16-5.24]) or were older (HR, 1.35 [1.00-1.83] per decade) had increased risk of malignancy, whereas female patients (HR, 0.37 [0.14-0.96]) and those with submandibular gland involvement (HR, 0.25 [0.10-0.61]) had decreased risk.
CONCLUSIONS: IgG4-RD was not associated with an increased incidence of malignancy following IgG4-RD onset. Among patients with IgG4-RD, smoking and older age at IgG4-RD onset were associated with higher malignancy risk. Female sex and submandibular gland involvement were associated with lower malignancy risk.