Malignancy Risk of Immunoglobin G4-Related Disease: Evidence from a Large Cohort Multicenter Retrospective Study

Abstract

Introduction: The aim of this work was to evaluate the prevalence of malignancies in a multicenter cohort of Chinese patients with immunoglobulin G4-related disease (IgG4-RD) and to identify the related risk factors of malignancy in IgG4-RD patients. Methods: We retrospectively analyzed 602 IgG4-RD patients who were recruited in five medical centers from 2009 to 2020. Standardized prevalence ratios (SPRs) against the general Chinese population were calculated along with 95% confidence intervals (CIs). We identified the risk factors of malignancy in IgG4-RD and calculated the odds ratios (ORs) of different factors. We then developed and validated a prediction model for malignancy risk of IgG4-RD based on our cohort. Results: We observed a significantly increased prevalence of total malignancies in this cohort compared to the general Chinese population (SPR 8.66 [95% CI 5.84, 12.31]). Logistic regression analysis indicated that eosinophil percentage (OR 1.096 [95% CI 1.019-1.179], P = 0.016), serum albumin-to-globulin ratio (AGR) (OR 0.185 [95% CI 0.061-0.567], P = 0.002) and autoimmune pancreatitis (OR 2.400 [95% CI 1.038-5.549], P = 0.041) were three potential risk factors of malignancy in IgG4-RD patients. Four predictors were included in our final prediction model: age at IgG4-RD diagnosis, eosinophil percentage, AGR and autoimmune pancreatitis. The nomogram performed well in the internal validation cohort, with a concordance index (C-index) of 0.738. Conclusions: A significantly increased prevalence of total malignancies was observed in our Yanying Liu and Jiangnan Fu contributed equally to this paper.

Comorbidities and causes of hospitalizations in a cohort of IgG4‐related disease patients from a single center

“…Herein,wefoundthatover90%ofpatientswithIgG4-RDhad at least one comorbidity, with the most prevalent being diabetes mellitus,hypertension,andatopy.Ourstudyshowedahigherprevalenceofdiabetes(47%)comparedwithpreviouslyreportedfigures (16.4%-39%). [6][7][8] We observed a higher prevalence of type 2 and type 3c diabetes in group 4, followed by group 1, which are the phenotypes in which pancreatic involvement is more prevalent. 3 Conversely, another study found diabetes to be more common in groups 1 and 4, despite higher cumulative doses of glucocorti-coidsingroup3.…”

“Stomach mass” as the first manifestation of IgG4-related disease: a case report

“…IgG4-related disease can create an immunological context that may impair the tumoral immunosurveillance and promote the pancreatic carcinogenesis [ 13 ]. Autoimmune pancreatitis was identifed as a potential risk factor for IgG4 related patients having malignancy [ 3 ]. Although autoimmune pancreatitis was not found in this patient, due to the potential for malignancy, physicians should be alert to this possibility during follow-up.…”

“…IgG4-related disease is immune-mediated chronic inflammation that is diagnosed by clinicopathological presentations of enlarged organs, elevation of serum concentrations of IgG4 and pathological findings such as a typical dense lymphoplasmacytic infiltration enriched in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis [ 1 , 2 ]. Pancreas, kidney, and retroperitoneum are often affected [ 3 ]. However, there are few reports of IgG4-related disease involving the stomach.This article analysed a patient with IgG4-related disease who had a “stomach tumor” as the initial manifestation.…”

Comment on “The risk of malignancy in patients with IgG4-related disease: a systematic review and meta-analysis” by Yu et al.

“…Second, IgG4-RD is a fibrous-inflammatory process that often involves multiple organs, either synchronously or metachronously, including cervical lymph nodes, lung, liver, bile duct, gastrointestinal tract, central nervous system, thyroid, pancreas, kidney, prostate, arteries, lymph nodes, skin, breast, lacrimal glands and salivary gland [ 5 ]. Although organ distribution varies among studies, the pancreas, bile ducts, and salivary glands are the most frequently involved organs [ 2 , 6 ]. However, malignant tumors related to IgG4-RD proposed in the study were only confined to four diseases (pancreatic cancer, lymphoma, gastric cancer, and lung cancer).…”