Outcome of Very Early Onset Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis: A Multicenter Study From the Pediatric IBD Porto Group of ESPGHAN.

IF 4.5 3区医学 Q1 GASTROENTEROLOGY & HEPATOLOGY Inflammatory Bowel Diseases Pub Date : 2024-10-03 DOI:10.1093/ibd/izad218

Giulia Catassi, Giulia D'Arcangelo, Lorenzo Norsa, Matteo Bramuzzo, Iva Hojsak, Kaija-Leena Kolho, Claudio Romano, Marco Gasparetto, Angelo Di Giorgio, Seamus Hussey, Anat Yerushalmy-Feler, Dan Turner, Manar Matar, Batia Weiss, Anna Karoliny, Patrizia Alvisi, Christos Tzivinikos, Marina Aloi

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Abstract

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.

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与原发性硬化性胆管炎相关的早期炎症性肠病的转归：ESPGHAN儿童IBD Porto组的多中心研究。

背景：6年前诊断的原发性硬化性胆管炎是否与炎症性肠病（PSC-IBD）有关（即VEO-IBD）具有不同的表型和病程尚未研究。我们旨在分析VEO-PSC-IBD的特征和自然史，并与早期和青少年发作的PSC-IBD.方法：这是一项多中心、回顾性、病例对照研究，来自ESPGHAN Porto和Interest IBD组的15个中心。在基线和每6个月收集一次人口学、临床、实验室、内窥镜和影像学数据。比较两组之间与炎症性肠病相关（临床缓解、需要全身类固醇和生物制剂以及手术）和PSC相关（胆道和门脉高压并发症、需要升级治疗和肝移植、胆管癌或死亡）的结果。结果：纳入69名儿童，中位随访时间为3.63年（四分位间距，1-11）：VEO-PSC-IBD 28名（23名UC[82%]，2名IBD-U[7%]和3名[11%]CD），PSC-IBB 41名（37名UC[90%]，3名IBDU[7.5%]和1名[2.5%]CD。大多数UC患者表现为泛结肠炎（VEO-PSC-UC中92%对PSC-UC的85%，P=.2）。与年龄较大的儿童相比，VEO-PSC-CBD患者被诊断为PSC/自身免疫性肝炎重叠综合征的人数更多（24[92%]对27[67.5%]PSC-IBD，P=.03），而PSC相关变量没有发现其他差异。VEO-PSC-IBD组发生胆道狭窄和感染性胆管炎的时间较低（分别为P=0.01和P=0.04），而其他结果无差异。无胆管癌病例报告。结论：与炎症性肠病相关的原发性硬化性胆管炎无论诊断为VEO-IBD还是之后，都具有相似的基线特征。VEO-PSC-IBD的特点是胆道并发症的病程较轻。

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来源期刊

Inflammatory Bowel Diseases 医学-胃肠肝病学

CiteScore

9.70

自引率

6.10%

发文量

462

审稿时长

1 months

期刊介绍： Inflammatory Bowel Diseases® supports the mission of the Crohn''s & Colitis Foundation by bringing the most impactful and cutting edge clinical topics and research findings related to inflammatory bowel diseases to clinicians and researchers working in IBD and related fields. The Journal is committed to publishing on innovative topics that influence the future of clinical care, treatment, and research.