Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group
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 incept...
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| Vydáno v: | Journal of pediatric gastroenterology and nutrition Ročník 80; číslo 2; s. 374 - 393 |
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| Hlavní autoři: | , , , , , , , , , , , , , , , , , , , , |
| Médium: | Journal Article |
| Jazyk: | angličtina |
| Vydáno: |
United States
01.02.2025
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| Témata: | |
| ISSN: | 0277-2116, 1536-4801, 1536-4801 |
| On-line přístup: | Získat plný text |
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| 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.
What is Known
•
Primary sclerosing cholangitis (PSC) is an uncommon condition in childhood‐onset inflammatory bowel disease (IBD) with severe outcomes, and there is no consensus on diagnostic and treatment approaches.
What is New
•
The European Society for Paediatric Gastroenterology, Hepatology and Nutrition Hepatology Committee and the Pediatric IBD Porto group formulated clinical questions considered to be relevant for the care of children with PSC‐IBD.
•
An evidence‐supported approach to diagnose, monitor, and treat children with PSC–IBD is presented here, as well as suggestions for future studies based on current knowledge gaps. |
|---|---|
| AbstractList | We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).OBJECTIVEWe aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).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.METHODSThe 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.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.RESULTSRegular 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.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.CONCLUSIONSWe 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. 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. What is Known • Primary sclerosing cholangitis (PSC) is an uncommon condition in childhood‐onset inflammatory bowel disease (IBD) with severe outcomes, and there is no consensus on diagnostic and treatment approaches. What is New • The European Society for Paediatric Gastroenterology, Hepatology and Nutrition Hepatology Committee and the Pediatric IBD Porto group formulated clinical questions considered to be relevant for the care of children with PSC‐IBD. • An evidence‐supported approach to diagnose, monitor, and treat children with PSC–IBD is presented here, as well as suggestions for future studies based on current knowledge gaps. We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC). 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. 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. 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. |
| Author | Indolfi, Giuseppe Nita, Andreia Kolho, Kaija‐Leena Pakarinen, Mikko Samyn, Marianne Deganello, Annamaria Deneau, Mark R. Rheenen, Patrick F. Nicastro, Emanuele Sonzogni, Aurelio Russell, Richard K. Hussey, Séamus Ridder, Lissy Hojsak, Iva Fitzpatrick, Emer Junge, Norman Tenca, Andrea De Laffolie, Jan Ricciuto, Amanda Aloi, Marina Griffiths, Anne M. |
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| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/39741383$$D View this record in MEDLINE/PubMed |
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| Copyright | 2024 The Author(s). published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. 2024 The Author(s). Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. |
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| Keywords | colorectal carcinoma surveillance colonoscopy ursodeoxycholic acid MRCP calprotectin |
| Language | English |
| License | Attribution-NonCommercial 2024 The Author(s). Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. |
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| Notes | CME module may be found at https://learnonline.naspghan.org/jpgn2 Marianne Samyn and Giuseppe Indolfi contributed equally and share the last authorship. ESPGHAN is not responsible for the practices of physicians and provides guidelines and position papers as indicators of best practice only. Diagnosis and treatment is at the discretion of the healthcare provider. Disclaimers Patrick F. van Rheenen, Kaija‐Leena Kolho, and Richard K. Russell contributed equally and share the first authorship. Although this paper is produced by the ESPGHAN Hepatology Committee and the IBD Porto Group it does not necessarily represent ESPGHAN policy and is not endorsed by ESPGHAN. ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
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| PublicationTitle | Journal of pediatric gastroenterology and nutrition |
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| PublicationYear | 2025 |
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We aimed to provide an evidence‐supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary... We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing... |
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| SubjectTerms | Adolescent calprotectin Child Cholangiopancreatography, Magnetic Resonance Cholangitis, Sclerosing - complications Cholangitis, Sclerosing - diagnosis Cholangitis, Sclerosing - etiology Cholangitis, Sclerosing - therapy colorectal carcinoma Gastroenterology Humans Inflammatory Bowel Diseases - complications MRCP surveillance colonoscopy ursodeoxycholic acid |
| Title | Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group |
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