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Sclerosing Cholangitis in Pediatric Inflammatory Bowel Disease: Early Diagnosis and Management Affect Clinical
Kai O Hensel1, Eirini Kyrana2, Nedim Hadzic3
1Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Cambridge University Hospitals NHS Foundation Trust, Addenbrooke's Hospital, Cambridge, United Kingdom; Department of Pediatrics, University of Cambridge, Cambridge, United Kingdom.
Insights
Children with inflammatory bowel disease (IBD) and sclerosing cholangitis (SC) show improved outcomes, especially with early diagnosis. Promptly assess for SC in pediatric IBD patients with liver function abnormalities.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Inflammatory Bowel Disease Research
Background:
- Inflammatory Bowel Disease (IBD) and Sclerosing Cholangitis (SC) can co-occur in pediatric patients.
- Understanding the clinical course and outcomes of IBD-SC in children is crucial for effective management.
- Previous reports suggest poorer outcomes for pediatric IBD-SC compared to adult populations.
Purpose of the Study:
- To characterize the clinical features and outcomes of pediatric patients diagnosed with IBD and SC.
- To compare the clinical course and management strategies between different pediatric IBD-SC cohorts.
- To identify factors influencing the clinical outcomes in children with IBD-SC.
Main Methods:
- Retrospective analysis of clinical data from two UK tertiary centers (pediatric gastroenterology and hepatology).
- Inclusion of pediatric patients diagnosed with IBD and SC.
- Evaluation of clinical characteristics, management, and patient outcomes over a defined follow-up period.
Main Results:
- Eighty-two pediatric patients with IBD-SC were analyzed (mean age at diagnosis 11.9 years).
- Ulcerative colitis was the most common IBD type (55%); Autoimmune SC (ASC) was diagnosed in 72%.
- Excellent 5- and 10-year complication-free (96%, 75%) and native liver survival (100%, 88%) rates were observed. Early diagnosis of liver disease post-IBD diagnosis correlated with no liver complications.
Conclusions:
- Pediatric patients with IBD-SC demonstrate better clinical outcomes than previously reported, particularly with early detection.
- Prompt evaluation for SC, including liver biopsy and imaging, is recommended for pediatric IBD patients with abnormal liver function tests.
- Early intervention and multidisciplinary management may contribute to improved outcomes in pediatric IBD-SC.
Objective:
To describe the characteristics and clinical course of children and young persons with inflammatory bowel disease (IBD) and sclerosing cholangitis (SC).
Study Design:
Retrospective analysis of clinical characteristics, management, and outcome of two separate cohorts of children and young persons with IBD-SC managed in a tertiary pediatric gastroenterology center and in a tertiary pediatric hepatology center in the UK.
Results:
Eighty-two pediatric patients (31% female) with IBD-SC and a mean age at diagnosis of 11.9 ± 2.8 years were followed up for a mean of 6.8 ± 3.3 years. The most common type of IBD was ulcerative colitis (55%), followed by unclassified IBD (30%) and Crohn's disease (15%). Autoimmune SC (ASC) was diagnosed in 72%, and small duct SC was diagnosed in 28%. Complication-free and native liver survival were 96% and 100%, respectively, at 5 years after diagnosis and 75% and 88%, respectively, at 10 years after diagnosis. Patients in the gastroenterology center, who were diagnosed with liver disease sooner after diagnosis of IBD compared with the hepatology center cohort (mean, 2.7 ± 6.1 months vs 9.3 ± 19.4 months; P = .03), did not develop liver-related complications during follow-up.
Conclusions:
Our data suggest that children with IBD-SC have better clinical outcomes than have been reported previously, particularly if diagnosed early. We recommend prompt assessment for SC, including liver biopsy and biliary imaging, when liver function abnormalities are detected in a children diagnosed with IBD.
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