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

Abstract

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.

Keywords: diagnosis; epidemiology; gastrointestinal tract; immunoglobulin G4-related disease; systematic review.

Figure 1

Identification flowchart of included studies. * number of papers found in the four databases.

Figure 2

Clinical manifestations of the digestive tract by IgG4-RD.

Figure 3

Typical features of AIP 2 in IgG4-RD seen on CT and MRI—Diffuse enlargement of the pancreas at CT (A,B) with blurring of peripancreatic fat (dashed yellow arrow, on B). T1-weighted MRI image of the pancreas (C, asterisk indicates pancreatic fibrosis) showing pancreatic atrophy (solid yellow arrow, D) [Adapted] [23].

Figure 4

Typical features of primary sclerosing cholangitis in IgG4-RD seen on CT and MRI—dilatation of the intrahepatic bile ducts on the left side (solid arrow, A) and cholangio-MRI image showing dilatation of the extrahepatic and intrahepatic bile ducts (left and right) with areas of stenosis (dashed yellow arrow, B) [Adapted] [23].

Figure 5

Typical features of retroperitoneal fibrosis in IgG4-RD seen on CT and MRI. Fibrotic process involving the ureters and iliac arteries in the abdomen (dashed mark, A) with hydronephrosis (asterisks, B). [Adapted] [23].