1 - Gastrenterology And Heptology Unit, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal
- Poster, 56th Annual Meeting of the European Society for Pediatric Gastroenterology, Hepatology and Nutricion (ESPGHAN), Milão, 15-18 de maio 2024
RESUMO:
Objectives and Study: Pediatric sclerosing cholangitis (SC) is a rare, chronic condition characterized by progressive inflammation and fibrosis of the bile ducts. It is frequently linked to inflammatory bowel disease (IBD), particularly ulcerative colitis (UC), or overlapping with autoimmune hepatitis (AIH). Over time, SC may progress to biliary cirrhosis, complications, and even cholangiocarcinoma. This study aimed to characterize a cohort of pediatric patients with SC.
Methods: A retrospective observational study was conducted on pediatric SC cases under follow-up in 2022 at a tertiary pediatric gastroenterology clinic. The diagnosis of SC was established through imaging and/or histological evidence of bile duct injury. Data collection encompassed demographic, laboratory, imaging, and histological information.
Results: Nineteen patients were included, with a median age at diagnosis of 12.5 years, predominantly female (52%).Median follow up into the clinic is 3,3 years (0,4-12,7). A family history of autoimmune disease was present in 21%. Most patients had a diagnosis of IBD (78%), 67% UC. Overlapping with AIH type 1 in 21%. No cases of SC not associated with IBD or AIH were recorded. At diagnosis, all patients had elevated GGT, AST, and ALT. Fifteen patients (79%) had autoantibodies (ANA 80%; pANCA 40%; ASCA 5%). Ultrasound changes suggestive of SC were found in 47%. Magnetic resonance cholangiopancreatography revealed bile duct ectasia in 42%. One patient with stenosis underwent stent placement with favorable evolution. Liver biopsy was performed in 63%. All patients were treated with ursodeoxycholic acid, two patients received oral vancomycin. No complications of portal hypertension, need for liver transplantation, or development of cholangiocarcinoma were observed.
Conclusions: This study underscores the connection between SC, IBD, and AIH in pediatric patients. While complications were absent in this cohort during the relatively short follow-up, the sustained surveillance of these patients is essencial to understand the dynamic nature of this association and ensuring timely intervention if complications arise.