Pediatr Gastroenterol Nutr. 2024 Dec 31. doi: 10.1002/jpn3.12378. Online ahead of print.

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

Author information

¹Department of Paediatric Gastroenterology, Hepatology, and Nutrition, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

²Children's Hospital, University of Helsinki and HUS, Helsinki, Finland.

³Department of Paediatric Gastroenterology, and Nutrition, Royal Hospital for Children and Young People, Edinburgh, UK.

⁴Sapienza University of Rome - Umberto I Hospital, Rome, Italy.

⁵Department of Radiology, King's College Hospital, School of Biomedical Engineering and Imaging Sciences, King's College London, London, UK.

⁶Children's Health Ireland and University College Dublin, Dublin, Ireland.

⁷Division for Pediatric Gastroenterology and Hepatology, Department of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School, Hannover, Germany.

⁸General Paediatrics and Neonatology, Gastroenterology, Justus Liebig University Giessen, Giessen, Germany.

⁹University of Utah and Intermountain Healthcare Primary Children's Hospital, Salt Lake City, Utah, USA.

¹⁰Faculty of Medicine, IBD Centre, SickKids Hospital, University of Toronto, Toronto, Ontario, Canada.

¹¹Children's Hospital Zagreb, University of Zagreb Medical School, Zagreb, Croatia.

¹²Pediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy.

¹³Department of Paediatric Gastroenterology, Great Ormond Street Hospital, London, UK.

¹⁴Department of Pediatric Surgery, The New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

¹⁵Department of Paediatric Gastroenterology, Erasmus University Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.

¹⁶Department of Pathology, ASST Bergamo Est, Bergamo, Italy.

¹⁷Helsinki University and Helsinki University Hospital HUS, Abdominal Center, Helsinki, Finland.

¹⁸Paediatric Liver, GI and Nutrition Service, King's College Hospital, London, UK.

¹⁹Meyer Children's Hospital IRCCS, Florence, Italy.

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.