TY - JOUR
T1 - Outcome of Very Early Onset Inflammatory Bowel Disease Associated with Primary Sclerosing Cholangitis
T2 - A Multicenter Study from the Pediatric IBD Porto Group of ESPGHAN
AU - Catassi, Giulia
AU - D'Arcangelo, Giulia
AU - Norsa, Lorenzo
AU - Bramuzzo, Matteo
AU - Hojsak, Iva
AU - Kolho, Kaija Leena
AU - Romano, Claudio
AU - Gasparetto, Marco
AU - Di Giorgio, Angelo
AU - Hussey, Seamus
AU - Yerushalmy-Feler, Anat
AU - Turner, Dan
AU - Matar, Manar
AU - Weiss, Batia
AU - Karoliny, Anna
AU - Alvisi, Patrizia
AU - Tzivinikos, Christos
AU - Aloi, Marina
N1 - Publisher Copyright:
© 2023 The Author(s). Published by Oxford University Press on behalf of Crohn's & Colitis Foundation. All rights reserved.
PY - 2024/10/1
Y1 - 2024/10/1
N2 - Background: Whether primary sclerosing cholangitis related to inflammatory bowel disease (PSC-IBD) diagnosed before 6 years (ie, VEO-IBD) has a distinct phenotype and disease course is uninvestigated. We aimed to analyze the characteristics and natural history of VEO-PSC-IBD, compared with early and adolescent-onset PSC-IBD. Methods: This is a multicenter, retrospective, case-control study from 15 centers affiliated with the Porto and Interest IBD group of ESPGHAN. Demographic, clinical, laboratory, endoscopic, and imaging data were collected at baseline and every 6 months. Inflammatory bowel disease-related (clinical remission, need for systemic steroids and biologics, and surgery) and PSC-related (biliary and portal hypertensive complications, need for treatment escalation and liver transplantation, cholangiocarcinoma, or death) outcomes were compared between the 2 groups. Results: Sixty-nine children were included, with a median follow-up of 3.63 years (interquartile range, 1-11): 28 with VEO-PSC-IBD (23 UC [82%], 2 IBD-U [7%] and 3 [11%] CD), and 41 with PSC-IBD (37 UC [90%], 3 IBDU [7.5%] and 1 [2.5%] CD). Most patients with UC presented with pancolitis (92% in VEO-PSC-UC vs 85% in PSC-UC, P =. 2). A higher number of patients with VEO-PSC-IBD were diagnosed with PSC/autoimmune hepatitis overlap syndrome than older children (24 [92%] vs 27 [67.5%] PSC-IBD, P =. 03), whereas no other differences were found for PSC-related variables. Time to biliary strictures and infective cholangitis was lower in the VEO-PSC-IBD group (P =. 01 and P =. 04, respectively), while no difference was found for other outcomes. No cases of cholangiocarcinoma were reported. Conclusions: Primary sclerosing cholangitis related to inflammatory bowel disease has similar baseline characteristics whether diagnosed as VEO-IBD or thereafter. A milder disease course in terms of biliary complications characterizes VEO-PSC-IBD.
AB - Background: Whether primary sclerosing cholangitis related to inflammatory bowel disease (PSC-IBD) diagnosed before 6 years (ie, VEO-IBD) has a distinct phenotype and disease course is uninvestigated. We aimed to analyze the characteristics and natural history of VEO-PSC-IBD, compared with early and adolescent-onset PSC-IBD. Methods: This is a multicenter, retrospective, case-control study from 15 centers affiliated with the Porto and Interest IBD group of ESPGHAN. Demographic, clinical, laboratory, endoscopic, and imaging data were collected at baseline and every 6 months. Inflammatory bowel disease-related (clinical remission, need for systemic steroids and biologics, and surgery) and PSC-related (biliary and portal hypertensive complications, need for treatment escalation and liver transplantation, cholangiocarcinoma, or death) outcomes were compared between the 2 groups. Results: Sixty-nine children were included, with a median follow-up of 3.63 years (interquartile range, 1-11): 28 with VEO-PSC-IBD (23 UC [82%], 2 IBD-U [7%] and 3 [11%] CD), and 41 with PSC-IBD (37 UC [90%], 3 IBDU [7.5%] and 1 [2.5%] CD). Most patients with UC presented with pancolitis (92% in VEO-PSC-UC vs 85% in PSC-UC, P =. 2). A higher number of patients with VEO-PSC-IBD were diagnosed with PSC/autoimmune hepatitis overlap syndrome than older children (24 [92%] vs 27 [67.5%] PSC-IBD, P =. 03), whereas no other differences were found for PSC-related variables. Time to biliary strictures and infective cholangitis was lower in the VEO-PSC-IBD group (P =. 01 and P =. 04, respectively), while no difference was found for other outcomes. No cases of cholangiocarcinoma were reported. Conclusions: Primary sclerosing cholangitis related to inflammatory bowel disease has similar baseline characteristics whether diagnosed as VEO-IBD or thereafter. A milder disease course in terms of biliary complications characterizes VEO-PSC-IBD.
KW - phenotype
KW - primary sclerosing cholangitis
KW - very early onset inflammatory bowel disease
KW - very early onset primary sclerosing cholangitis
UR - http://www.scopus.com/inward/record.url?scp=85205603618&partnerID=8YFLogxK
U2 - 10.1093/ibd/izad218
DO - 10.1093/ibd/izad218
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C2 - 37768032
AN - SCOPUS:85205603618
SN - 1078-0998
VL - 30
SP - 1662
EP - 1669
JO - Inflammatory Bowel Diseases
JF - Inflammatory Bowel Diseases
IS - 10
ER -
