Gastroenterological Manifestations of Immunoglobulin G Subclass 4-Related Disease-Epidemiology, Clinical Manifestations, Diagnosis and Treatment

Despite causing high morbidity, IgG4-related disease (IgG4-RD) and its gastroenterological manifestations lack better and greater theoretical contributions. Therefore, the objective of this work was to evaluate the clinical-epidemiological, diagnostic and treatment aspects of the gastrointestinal manifestations of this disease. A systematic review was carried out using the PubMed, Scopus and Embase databases between January 2012 and March 2023 with the following descriptors: Immunoglobulin G4-Related Disease (MeSH) AND #2 Gastrointestinal Tract (MeSH). Our data collection grouped a total of 3607 patients from mostly epidemiological cohort studies and cross-sectional follow-ups. In the subgroup analysis, IgG4-RD was associated with male gender, with an estimated prevalence between 54% and 80%. In our findings, the prevalence by topography was presented in the following ranges: lacrimal glands (17-57%); salivary glands (28-72%); pancreas (19-60%); biliary tract (5-40%); retroperitoneal cavity (9-43%). Longitudinal studies are needed to better map the natural history of the gastrointestinal manifestations of IgG4-RD and enable the formulation of individualized treatments.

Automated summary

Despite the high morbidity of IgG4-related disease (IgG4-RD) and its gastrointestinal manifestations, there is a lack of substantial theoretical contributions. This study aimed to evaluate the clinical-epidemiological, diagnostic, and treatment aspects of the gastrointestinal manifestations of IgG4-RD. A systematic review of relevant databases was conducted, and the findings showed a predominance of male gender and varying prevalence rates in different anatomical locations.