未分类的儿童炎症性肠病：在苏格兰进行的一项长达20年随访的全国性队列研究表明，大多数病例会重新分类，而诊断未改变的病例病程较轻。

Pediatric Inflammatory Bowel Disease Type Unclassified: A Nationwide Cohort Study in Scotland With up to 20 Years Follow-up Shows Reclassification in the Majority and Mild Course in Those Whose Diagnosis Is Unchanged.

作者信息

Wands David I F, Gianolio Laura, Cameron Fiona, Hansen Richard, Russell Richard K, Wilson David C

机构信息

Child Life and Health, University of Edinburgh, Royal Hospital for Children & Young People, Edinburgh, UK.

Department of Paediatric Gastroenterology, Hepatology and Nutrition, Royal Hospital for Children, Glasgow, UK.

出版信息

Inflamm Bowel Dis. 2025 Feb 10;31(2):313-320. doi: 10.1093/ibd/izae218.

DOI:10.1093/ibd/izae218

PMID:39321100

Abstract

BACKGROUND

Given the paucity of long-term longitudinal data for inflammatory bowel disease type unclassified (IBDU), we aimed to clarify IBDU disease course and reclassification rate by presenting nationwide data with up to 20 years of follow-up.

METHODS

We analyzed a prospectively identified 11-year cohort of pediatric patients diagnosed with IBDU between January 1, 2003 and December 31, 2013 at all Scottish pediatric IBD centers and followed up into adult services until December 31, 2022. Data were obtained from electronic medical records at fixed timepoints (5 and 10 years post-diagnosis) and at the final follow-up.

RESULTS

Overall, 102 patients were included in the analysis (57/102 [56%] male, median [interquartile range {IQR}] age at diagnosis: 11.5 [9.1-13.2] years) with a median (IQR) follow-up length of 10.5 (8.6-14.0) years. A change of diagnosis was made in 61 of 102 patients (60%); of these, 30 patients (29%) were reclassified to Crohn's disease (CD) and 31 patients (30%) to ulcerative colitis (UC). Patients who remained with IBDU had higher 1- to 5-year remission rates (IBDU 30/39 [77%] vs reclassified 16/57 [28%], P < .05), with lower rates of moderate-to-severe disease (IBDU 3/39 [8%] vs reclassified 31/57 [54%], P < .05) and less need for biologics across all timepoints (IBDU vs reclassified: first timepoint 1/39 [3%] vs 17/57 [30%], second timepoint 1/33 [3%] vs 26/56 [46%], third timepoint 0/18 [0%] vs 16/33 [49%]; all P < .05). Higher rates of surgical resections were observed in reclassified patients (reclassified 11/61 [18%] vs IBDU 1/41 [2%], P = .02).

CONCLUSIONS

In our nationwide pediatric IBDU cohort, 60% of patients were reclassified to either UC or CD over 10.5 years of median follow-up; those who remained with IBDU had a milder disease course.

摘要

背景

鉴于未分类的炎症性肠病（IBDU）长期纵向数据匮乏，我们旨在通过呈现长达20年随访的全国性数据，阐明IBDU的疾病进程和重新分类率。

方法

我们分析了一个前瞻性确定的队列，该队列中的儿科患者于2003年1月1日至2013年12月31日在苏格兰所有儿科炎症性肠病中心被诊断为IBDU，并随访至成人服务阶段，直至2022年12月31日。数据在固定时间点（诊断后5年和10年）以及最终随访时从电子病历中获取。

结果

总体而言，102例患者纳入分析（57/102 [56%]为男性，诊断时年龄中位数[四分位间距{IQR}]：11.5 [9.1 - 13.2]岁），随访时间中位数（IQR）为10.5（8.6 - 14.0）年。102例患者中有61例（60%）诊断发生了变化；其中，30例患者（29%）重新分类为克罗恩病（CD），31例患者（30%）重新分类为溃疡性结肠炎（UC）。仍诊断为IBDU的患者1至5年缓解率更高（IBDU为30/39 [77%]，重新分类的为16/57 [28%]，P < 0.05），中重度疾病发生率更低（IBDU为3/39 [8%]，重新分类的为31/57 [54%]，P < 0.05），且在所有时间点使用生物制剂的需求更少（IBDU与重新分类的患者相比：第一个时间点1/39 [3%]对17/57 [30%]，第二个时间点1/33 [3%]对26/56 [46%]，第三个时间点0/18 [0%]对16/33 [49%]；所有P < 0.05）。重新分类的患者手术切除率更高（重新分类的为11/61 [18%]，IBDU为1/41 [2%]，P = 0.02）。