Abstract

BackgroundThe relationship between IgG4-related disease (IgG4-RD) and the risk of malignancy is still controversial. This article focused on assessing the risk of cancer in patients with IgG4-RD by meta-analysis.MethodsWe conducted a systematic review of the literature and meta-analysis characterizing the associated risk of overall malignancy and four site-specific malignancies (pancreas, lung, gastric and lymphoma) in patients with IgG4-RD. A search from 2003 to 2020 was performed using specified terms from PubMed, Embase, Web of Science and SinoMed. Random-effects model analysis was used to pool standardized incidence ratios (SIRs) and 95% confidence intervals (CIs). Subgroup and sensitivity analyses were conducted to clarify the heterogeneity of the included studies. Begg’s funnel plot and Egger’s linear regression test were used to evaluate the bias of the meta-analysis. A P value < 0.05 indicated the existence of publication bias.ResultsA total of 10 studies were included in the article. The overall SIR estimates suggested an increased risk of overall cancer in IgG4-RD patients (SIR 2.57 95% CI 1.72–3.84) compared with the general population. The specific SIRs for pancreas and lymphoma were higher than those of the general population in IgG4-RD patients (SIR 4.07 95% CI 1.04–15.92, SIR 69.17 95% CI 3.91–1223.04, respectively). No significant associations were revealed in respiratory and gastric cancer (SIR 2.14 95% CI 0.97–4.75, SIR 0.95 95% CI 0.24–3.95, respectively). Four studies were found to be the major sources of heterogeneity by sensitivity analysis. There was no evidence of publication bias via Egger’s test.ConclusionCompared with the general population, patients with IgG4-RD appear to have a higher risk of overall cancer, especially pancreatic and lymphoma. The risk of lung and gastric cancer was not different between IgG4-RD patients and the general population.

Highlights

  • IgG4-related disease (IgG4-RD), featuring dense lymphoplasmacytic IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis in histopathology, is a chronicThe risk of malignancies in patients with IgG4-RD has not been clarified

  • Our meta-analysis revealed an association between IgG4-RD and increased cancer risk compared with the general population

  • Our meta-analysis presented a significant association between lymphoma and IgG4-RD

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Summary

Introduction

The risk of malignancies in patients with IgG4-RD has not been clarified. It should be noted that with ongoing further research, the initiation of immunomodulatory treatment and biologic agents has a potential therapeutic risk of increasing the incidence of malignancy by altering the normal function of immunosurveillance in. Understanding the baseline risk of malignancy is of great importance in patients with IgG4-RD. There has been no meta-analysis evaluating the risk of malignancy in IgG4-RD. We review the reported data on the incidence of malignancy and perform a systematic review and meta-analysis to assess the risk of malignant disease. The relationship between IgG4-related disease (IgG4-RD) and the risk of malignancy is still controversial. This article focused on assessing the risk of cancer in patients with IgG4-RD by meta-analysis

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