Clinical Features and Natural History of Paediatric Patients with Ulcerative Proctitis: A Multicentre Study from the Paediatric IBD Porto Group of ESPGHAN.

IF 8.3 2区医学 Q1 GASTROENTEROLOGY & HEPATOLOGY

Journal of Crohns & Colitis Pub Date : 2023-12-30 DOI:10.1093/ecco-jcc/jjad111

Noa Tal, Christos Tzivinikos, Marco Gasparetto, Daniela E Serban, Eyal Zifman, Iva Hojsak, Oren Ledder, Anat Yerushalmy Feler, Helena Rolandsdotter, Marina Aloi, Matteo Bramuzzo, Stephan Buderus, Paolo Lionetti, Lorenzo Norsa, Christoph Norden, Darja Urlep, Claudio Romano, Ron Shaoul, Christine Martinez-Vinson, Anna Karoliny, Elisabeth De Greef, Ben Kang, Eva VIčková, Patrizia Alvisi, Michal Kori, Marta Tavares, Batia Weiss, Seamus Hussey, Maria E Qamhawi, Laura M Palomino Pérez, Paul Henderson, Raj Parmar, Erasmo Miele, Firas Rinawi, Ana Lozano-Ruf, Veena Zamvar, Kaija-Leena Kolho, Dror S Shouval

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引用次数: 0

Abstract

Background and aims: Ulcerative proctitis [UP] is an uncommon presentation in paediatric patients with ulcerative colitis. We aimed to characterize the clinical features and natural history of UP in children, and to identify predictors of poor outcomes.

Methods: This was a retrospective study involving 37 sites affiliated with the IBD Porto Group of ESPGHAN. Data were collected from patients aged <18 years diagnosed with UP between January 1, 2016 and December 31, 2020.

Results: We identified 196 patients with UP (median age at diagnosis 14.6 years [interquartile range, IQR 12.5-16.0]), with a median follow-up of 2.7 years [IQR 1.7-3.8]. The most common presenting symptoms were bloody stools [95%], abdominal pain [61%] and diarrhoea [47%]. At diagnosis, the median paediatric ulcerative colitis activity index [PUCAI] score was 25 [IQR 20-35], but most patients exhibited moderate-severe endoscopic inflammation. By the end of induction, 5-aminosalicylic acid administration orally, topically or both resulted in clinical remission rates of 48%, 48%, and 73%, respectively. The rates of treatment escalation to biologics at 1, 3, and 5 years were 10%, 22%, and 43%, respectively. In multivariate analysis, the PUCAI score at diagnosis was significantly associated with initiation of systemic steroids, or biologics, and subsequent acute severe colitis events and inflammatory bowel disease-associated admission, with a score ≥35 providing an increased risk for poor outcomes. By the end of follow-up, 3.1% of patients underwent colectomy. Patients with UP that experienced proximal disease progression during follow-up [48%] had significantly higher rates of a caecal patch at diagnosis and higher PUCAI score by the end of induction, compared to those without progression.

Conclusion: Paediatric patients with UP exhibit high rates of treatment escalation and proximal disease extension.

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儿童溃疡性直肠炎患者的临床特征和自然史：ESPGHAN儿科IBD波尔图小组的一项多中心研究。

背景和目的：溃疡性直肠炎（UP）是溃疡性结肠炎儿科患者中一种不常见的表现。我们旨在描述儿童溃疡性直肠炎的临床特征和自然病史，并确定不良预后的预测因素：这是一项回顾性研究，涉及隶属于ESPGHAN IBD波尔图小组的37个研究机构。方法：这是一项回顾性研究，涉及隶属于ESPGHAN IBD波尔图小组的37个研究机构：我们发现了196名UP患者（确诊时的中位年龄为14.6岁[四分位间差为12.5-16.0]），中位随访时间为2.7年[四分位间差为1.7-3.8]。最常见的首发症状是血便[95%]、腹痛[61%]和腹泻[47%]。确诊时，小儿溃疡性结肠炎活动指数[PUCAI]的中位数为 25 [IQR 20-35]，但大多数患者表现为中度-重度内镜炎症。在诱导治疗结束时，口服、外用或同时服用 5- 氨基水杨酸可使临床缓解率分别达到 48%、48% 和 73%。1年、3年和5年后升级为生物制剂治疗的比例分别为10%、22%和43%。在多变量分析中，诊断时的 PUCAI 评分与开始使用全身类固醇或生物制剂以及随后的急性重症结肠炎事件和炎症性肠病相关入院治疗显著相关，评分≥35 分会增加不良预后的风险。随访结束时，3.1%的患者接受了结肠切除术。在随访期间出现近端疾病进展的UP患者[48%]与未出现进展的患者相比，在诊断时出现盲肠补片的比例明显更高，在诱导结束时PUCAI评分也更高：结论：UP 儿童患者的治疗升级率和近端疾病扩展率都很高。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Crohns & Colitis 医学-胃肠肝病学

CiteScore

15.50

自引率

7.50%

发文量

1048

审稿时长

1 months

期刊介绍： Journal of Crohns and Colitis is concerned with the dissemination of knowledge on clinical, basic science and innovative methods related to inflammatory bowel diseases. The journal publishes original articles, review papers, editorials, leading articles, viewpoints, case reports, innovative methods and letters to the editor.