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Practice Guideline
. 2025 Feb;80(2):374-393.
doi: 10.1002/jpn3.12378. Epub 2024 Dec 31.

Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group

Patrick F van Rheenen  1 , Kaija-Leena Kolho  2 , Richard K Russell  3 , Marina Aloi  4 , Annamaria Deganello  5 , Séamus Hussey  6 , Norman Junge  7 , Jan De Laffolie  8 , Mark R Deneau  9 , Emer Fitzpatrick  6 , Anne M Griffiths  10 , Iva Hojsak  11 , Emanuele Nicastro  12 , Andreia Nita  13 , Mikko Pakarinen  14 , Amanda Ricciuto  10 , Lissy de Ridder  15 , Aurelio Sonzogni  16 , Andrea Tenca  17 , Marianne Samyn  18 , Giuseppe Indolfi  19
Affiliations
Practice Guideline

Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group

Patrick F van Rheenen et al. J Pediatr Gastroenterol Nutr. 2025 Feb.

Abstract

Objective: We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).

Methods: The core group formulated seven PICO-structured clinical questions. A systematic literature search from inception to December 2022 was conducted by a medical librarian using MEDLINE and EMBASE. Core messages from the literature were phrased as position statements and then circulated to a sounding board composed of international experts in pediatric gastroenterology and hepatology, histopathology, adult gastroenterology and hepatology, radiology, and surgery. Statements reaching at least 80% agreement were considered as final. The other statements were refined and then subjected to a second online vote or rejection.

Results: Regular screening for gamma-glutamyltransferase (GGT) is essential for detecting possible biliary disease in children with IBD. MR cholangiopancreatography is the radiological modality of choice for establishing the diagnosis of PSC. Liver biopsy is relevant in the evaluation of small duct PSC or autoimmune hepatitis. Children who do not have known IBD at the time of PSC diagnosis should undergo initial screening with fecal calprotectin for asymptomatic colitis, and then at least once yearly thereafter. Children with a cholestatic liver enzyme profile can be considered for treatment with ursodeoxycholic acid and can continue if there is a meaningful reduction or normalization in GGT. Oral vancomycin may have a beneficial effect on GGT and intestinal inflammation, but judicious use is recommended due to the lack of long-term studies. Children with PSC-IBD combined with convincing features of autoimmune hepatitis may benefit from corticosteroids and antimetabolites.

Conclusions: We present state-of-the-art guidance on the diagnostic criteria, follow-up strategies, and therapeutic strategies and point out research gaps in children and adolescents with PSC-IBD.

Keywords: MRCP; calprotectin; colorectal carcinoma; surveillance colonoscopy; ursodeoxycholic acid.

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Conflict of interest statement

Patrick F. van Rheenen: Research Grant—European Crohn & Colitis Organisation. Leadership Role—Chair of Dutch PIBD Guideline (2022‐2024). Receipt of Donated Equipment—BÜHLMANN Laboratories AG. Kaija‐Leena Kohlo: Grants or Contracts: Helsinki University Hospital Research Fund—ongoing research projects on fecal microbiome; Pediatric Research Foundation (Finland)—ongoing research project on pediatric IBD Consultancy fees: Abbvie; Blocodex. Richard K. Russell: Grant/Contract: Nestle Health Science—ongoing; Ferring—past. Consultancy fees: Celtrion and Lily. Participated in Data Monitoring Committee: OCEAN Trial. Medical Writing: Pfizer. Honoraria Lecture: Janssen. Payment for Meeting attendance: Abbvie and Celltrion. Marina Aloi: Honoraria for Lectures—Takeda, Nestle. Safety Monitoring Board—Pfizer. Annamaria Deganelloe: none. Séamus Hussey: Research Grant—Janssen. Norman Jung: Speaker at annual meeting/HEPCOM member—ESPGHAN. Data Monitoring Committee—ERN Rare Liver member for Rare Liver Registry. Leadership Roles—ERN Rare Liver, ERN Transplant Child. Jan De Laffolie: Consulting Fees—Mirum, Takeda, Sanofi. Lecture Honoraria—Mirum, Baxter, Sanofi. Safety monitoring board member—Sanofi. Advisor to patient organizations—DCCV, DZG. Mark R. Deneaui: none. Emer Fitzpatrick: Grant/Contract—Health Research Board, Ireland (author's institution). Board member—ESPGHAN. Anne M. Griffiths: Grants/Research Support—Abbvie. Member of advisory board—Abbvie, Janssen, Lily. Honoraria for Lectures—Abbvie, Janssen, Nestle. Honoraria for Consultation—Abbvie, Amgen, Bristol Meyers Squib, Pfizer, Merck, Janssen, Lily. Iva Hojsak: Consultancy Fees—Abbot and Biocodex. Honoraria for Lectures—Sandoz, Nestle, BioGaia, Hipp, GM Pharma. Emanuele Nicastro: Served as member on advisory board—Mirum Pharmaceuticals. Andreia Nita: none. Mikko Pakarinen: none Amanda Ricciuto: none Lissy de Ridder: Collaboration (such as involved in industry‐sponsored studies, investigator‐initiated study, consultancy) with Abbvie, Eli Lilly, Takeda, Janssen, Medtronic and Pfizer. Aurelio Sonzogni: none Andrea Tenca: none Marianne Samyn: none Giuseppe Indolfi: Payment/honorarium Kedrion Pharma 2020.

Figures

Figure 1
Figure 1
Images from a magnetic resonance cholangiopancreatography (A) and an endoscopic retrograde cholangiopancreatography (B) taken from the same patient with primary sclerosing cholangitis demonstrating irregular intrahepatic and extrahepatic bile ducts. (Courtesy of Dr. Patrick van Rheenen, University Medical Center Groningen, The Netherlands).
Figure 2
Figure 2
Hematoxylin and eosin stained pathology slides. (A) is taken from a patient with primary sclerosing cholangitis (PSC) showing periductal onion skin fibrosis. (B) is taken from a patient with autoimmune hepatitis (AIH) showing an infiltrate of plasma cells and lymphocytes extending beyond the limiting plate. (C) is taken from a patient with PSC and features of AIH showing bile duct injury and interface hepatitis. Bar = 50 µm. (Courtesy of Dr. Marius van den Heuvel, histopathologist, University Medical Center Groningen, The Netherlands).
Figure 3
Figure 3
Conceptual diagnostic model for autoimmune liver disease in children with inflammatory bowel disease. AIH, autoimmune hepatitis; MRCP, magnetic resonance cholangiopancreatography; PSC, primary sclerosing cholangitis.
Figure 4
Figure 4
The IBD phenotype of PSC–IBD. IBD, inflammatory bowel disease; PSC, primary sclerosing cholangitis.
Figure 5
Figure 5
Summary of CRC‐free survival in patients with PSC and IBD at 5 and 10 years after diagnosis. CRC, colorectal cancer; IBD, inflammatory bowel disease; PSC, primary sclerosing cholangitis.
Figure 6
Figure 6
Summary flowchart of diagnosis, monitoring and treatment. The numbers between brackets refer to the relevant sections. AIH, autoimmune hepatitis; ALT, alanine aminotransferase; CRC, colorectal cancer; FC, fecal calprotectin; GGT, gamma‐glutamyltransferase; IBD, inflammatory bowel disease; MRCP, magnetic resonance cholangiopancreatography; PSC, primary sclerosing cholangitis; UDCA, ursodeoxycholic acid; ULN, upper limit of normal.
See this image and copyright information in PMC

References

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