Pacientes pediátricos com doença inflamatória intestinal e manifestações hepatobiliares e pancreáticas - uma série de casos

Abstract

Introduction: Inflammatory bowel disease (IBD) is a chronic immune-mediated condition that primarily affects the gastrointestinal tract but is frequently associated with extraintestinal manifestations, including hepatobiliary and pancreatic involvement. Methods: This descriptive and observational study, designed as a case series with a literature review, included children diagnosed with IBD and hepatobiliary and/or pancreatic manifestations followed at a paediatric gastroenterology service between 2020 and 2025. Results: During the study period, 214 patients with IBD were followed, including 166 (77.6%) with Crohn’s disease, 39 (18.2%) with ulcerative colitis, and 9 (4.2%) with IBD unclassified. Nine patients (4.2%) presented hepatobiliary and/or pancreatic alterations, with age at first diagnosis ranging from 4 to 13 years (mean 8 years, standard deviation 3.7 years). Initial IBD symptoms included haematochezia (100%), abdominal pain (77.8%), and weight loss and/or diarrhoea (66.7%). Hepatobiliary and pancreatic manifestations included primary sclerosing cholangitis (PSC) in six patients (66.6%), PSC/autoimmune hepatitis overlap in two patients, and acute pancreatitis in one patient. The interval between IBD and hepatobiliary/pancreatic diagnoses ranged from 0 to 60 months (median 5 months, interquartile range 36 months). Seven patients (77.7%) were symptomatic, and all presented laboratory and/or imaging findings consistent with the diagnoses. Conclusion: Paediatric IBD is a multisystemic disease, with hepatobiliary and pancreatic alterations occurring in 4.2% of patients. These manifestations may be present at diagnosis, precede it, or develop during the course of IBD, highlighting the importance of systematic laboratory and imaging evaluation for early detection and management.