Annals of the rheumatic diseases
October 8, 2026 · via Europe PMC
IgG4-related disease is not associated with an increased incidence of malignancy in a large, single-centre cohort.
Michelson DA, McMahon GA, Jha I, +5 more, Katz G.
AI summary — for doctors, generated from the abstract only
In a large single-centre cohort, IgG4-related disease was not associated with an increased incidence of malignancy compared with the general US population.
What was studied
Retrospective cohort of 348 patients meeting ACR/EULAR IgG4-RD criteria, comparing age- and sex-stratified cancer incidence with population rates and using Cox models to find associated characteristics.
Key findings
- 34 patients (10%) developed incident malignancy over a median 8.04 years, and the standardised incidence ratio was 0.88 (0.61-1.23)
- Ever-smoking (adjusted HR 2.46) and older age (HR 1.35 per decade) increased risk
- Female sex (HR 0.37) and submandibular gland involvement (HR 0.25) were associated with lower risk
Clinical relevance
Offers reassurance that IgG4-RD itself does not appear to raise cancer risk, so no special screening is implied, while standard risk factors still matter.
Who should care
Limitations
Single tertiary centre with malignancy partly collected by survey, small numbers for subgroup hazard ratios with wide intervals, and possible selection and survivor bias.
IgG4-RD did not show excess cancer risk, with smoking and age remaining the main risk factors.
This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.
Abstract
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.
DOI
10.1016/j.ard.2026.08.012
PMID
42850159
Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.
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