Unraveling the Complexities of Concomitant IBD and Hepatobiliary Disease, With Natalia Nedelkopoulou, MD

New research is shedding light on the complexity of managing children and young adults with concomitant inflammatory bowel disease (IBD) hepatopancreatobiliary (HPB) disease, highlighting ulcerative colitis (UC)/primary sclerosing cholangitis (PSC) as an especially high-risk subtype.1

Findings from the retrospective analysis of patients diagnosed with IBD and HPB disease before 16 years of age with a follow-up period up to 30 years of age were presented at the 2025 North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) Annual Meeting by Natalia Nedelkopoulou, MD, a consultant pediatric gastroenterologist at Sheffield Children's Hospital.

“The combination of inflammatory bowel disease and primary sclerosing cholangitis has been well described and established in the literature, and oftentimes these families come across very upsetting information on the internet about the natural history of primary sclerosing cholangitis,” Nedelkopoulou told HCPLive. “They come to our clinics looking for answers about the long term outcomes, the morbidity and mortality of this condition.”

IBD and concomitant HPB disease diagnosed in childhood have been considered to have a milder IBD phenotype, but paradoxically are associated with increased risk of HPB malignancy and colorectal cancer later in life.2 To better characterize these patients and their long-term outcomes, Nedelkopoulou and colleagues retrospective analyzed data for patients ≤ 16 years of age diagnosed with concomitant IBD and HPB disease, with a follow-up period up to 30 years of age, at a linked pediatric-adult center with a structured transition program.

A total of 38 patients with IBD and concomitant HPB disease were identified, with a mean age at diagnosis of 17.2 years (range, 5-30 years). Among the cohort, 63.2% had a primary diagnosis of IBD, 26.3% presented with simultaneous IBD and HPB, and 10.5% had a primary HPB diagnosis. The mean time interval between diagnoses was 17.5 months (0-144).

Investigators noted UC was the most common IBD subtype (68.4%), whereas concomitant HPB disease included PSC (65.8%), autoimmune hepatitis (23.7%), and autoimmune sclerosing cholangitis (10.5%). Of note, UC/PSC was the most common combination, affecting 50% of patients.

Biologic treatment for IBD was required in 65.8% of patients, of whom 34.2% required a second biologic and 24% of these required further escalation. Infliximab was the most common primary biologic, with vedolizumab the most common second line agent. JAK-inhibitors were used in 18.4% of patients, and 34.2% achieved clinical remission with Azathioprine or 5-ASA alone. Investigators pointed out 5.26% of patients with UC/PSC required a subtotal colectomy, and there were no cases of colorectal cancer.

In the 19 UC/PSC subtype patients, 3 (15.8%) patients developed HPB malignancy; 2 had intrahepatic cholangiocarcinoma, and 1 had gallbladder cancer. All 3 patients underwent extensive surgical resection but developed recurrence within 3 years. Palliative systemic therapy was offered, and 1 patient died aged 29 years.

Non-malignant outcomes include stage 4 liver fibrosis in 1 patient with UC/autoimmune hepatitis and liver transplantation in another patient with IBD-U/PSC. IBD/autoimmune hepatitis/autoimmune sclerosing cholangitis subtypes were treated with prednisolone (61.5%), azathioprine (76.9%) or mycophenolate mofetil (15.4%). Among the cohort, investigators noted patients did not develop significant portal hypertension.

“Our study really highlighted how important it is to have very structured and multidisciplinary transition programs,” Nedelkopoulou said. “We feel that it's of utmost importance to have these programs in order to help young patients engage with the adult services and also improve the clinical outcomes long term.”

Editors’ Note: Nedelkopoulou reports no relevant disclosures.

References

1. Bruce S, Inam F, Hubbard R, et al. LONG-TERM OUTCOMES OF COMBINED INFLAMMATORY BOWEL DISEASE AND HEPATOBILIARY DISEASE IN CHILDREN AND YOUNG ADULTS: A 10 YEAR EXPERIENCE. Presented at 2025 NASPGHAN Annual Meeting. November 5-9, 2025. Chicago, Illinois.

2. Fousekis FS, Theopistos VI, Katsanos KH, et al. Hepatobiliary Manifestations and Complications in Inflammatory Bowel Disease: A Review. Gastroenterology Research. https://doi.org/10.14740/gr990w