BackgroundPancreatitis and exocrine pancreatic insufficiency may occur as extraintestinal manifestations of inflammatory bowel disease. Recently, autoimmune pancreatitis and colitis have been described as presentations of IgG4-related disease. IgG4+ plasma cells have been identified in colon tissue from patients with refractory forms of inflammatory bowel disease. The presence of elevated serum/tissue levels of IgG4 and the frequency of exocrine pancreatic insufficiency in inflammatory bowel disease are still a source of controversy. Our aim was to investigate the meaning of elevated IgG4 levels in patients with inflammatory bowel disease.MethodsA cross-sectional study analyzed 56 patients with a diagnosis of inflammatory bowel disease recruited by convenience sampling from two tertiary centers in Midwestern Brazil. All patients underwent fecal pancreatic elastase testing for detection of exocrine pancreatic insufficiency and serum IgG4 measurement. Findings were correlated with clinical and epidemiological data and disease activity.ResultsElevated serum IgG4 levels were found in 10 patients, and were most frequent in ulcerative colitis (nine cases), with a prevalence ratio of 16.42 (95% CI: 3.32 - 79.58). Ten patients (10 of 56, 17.8%) were diagnosed with exocrine pancreatic insufficiency, which did not correlate with disease activity, and serum IgG4 levels.ConclusionExocrine pancreatic insufficiency is prevalent in patients with inflammatory bowel disease, but it is not associated with elevated serum IgG4 levels. The high prevalence of elevated serum IgG4 in ulcerative colitis suggests that this parameter has potential for use as a diagnostic biomarker.

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Immunoglobulin G4-related disease is a multisystem disorder with unique gastrointestinal tract manifestations, often simulating neoplasms and other inflammatory conditions. Appropriate clinical suspicion and application of internationally validated criteria can assist in making the proper diagnosis. This article describes two cases of patients presenting with biliary tract manifestations simulating lymphoproliferative disease and adenocarcinoma, respectively. Clinical, radiological, and histopathological findings ultimately led to the correct diagnosis, and revealed useful nuances for detection of future cases. Application of specific criteria, such as the classic Japan Biliary Association clinical diagnostic criteria published in 2012 and revised in 2020 as well as the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria, has limitations but provides important warnings to be considered in the diagnostic journey of these challenging cases.

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