儿童炎症性肠病（IBD）亚型包括未分类IBD的诊断标准的制定与验证：欧洲儿科胃肠病、肝病和营养学会（ESPGHAN）儿科IBD波尔图组的多中心研究

Development and Validation of Diagnostic Criteria for IBD Subtypes Including IBD-unclassified in Children: a Multicentre Study From the Pediatric IBD Porto Group of ESPGHAN.

作者信息

Birimberg-Schwartz Liron, Zucker David M, Akriv Amichay, Cucchiara Salvatore, Cameron Fiona L, Wilson David C, Lazowska Iza, Yianni Lambri, Paul Siba Prosad, Romano Claudio, Kolacek Sanja, Buderus Stephan, Pærregaard Anders, Russell Richard K, Escher Johanna C, Turner Dan

机构信息

Institute of Pediatric Gastroenterology, Shaare Zedek Medical Centre, Jerusalem, Israel.

Department of Statistics, Hebrew University of Jerusalem, Jerusalem, Israel.

出版信息

J Crohns Colitis. 2017 Sep 1;11(9):1078-1084. doi: 10.1093/ecco-jcc/jjx053.

DOI:10.1093/ecco-jcc/jjx053

PMID:28430891

Abstract

BACKGROUND

The revised Porto criteria identify subtypes of paediatric inflammatory bowel diseases: ulcerative colitis [UC], atypical UC, inflammatory bowel disease unclassified [IBDU], and Crohn's disease [CD]. Others have proposed another subclassifiction of Crohn's colitis. In continuation of the Porto criteria, we aimed to derive and validate criteria, termed "PIBD-classes," for standardising the classification of the different IBD subtypes.

METHODS

This was a multicentre retrospective longitudinal study from 23 centres affiliated with the Port -group of ESPGHAN. Both a hypothesis-driven judgmental approach and mathematical classification and regression tree [CART] modelling were used for creating a diagnostic algorithm. Since small bowel inflammation is easily recognised as CD, we focused here primarily on the phenotype of colitis.

RESULTS

In all, 749 IBD children were enrolled: 236 [32%] Crohn's colitis, 272 [36%] UC and 241 [32%] IBDU [age 10.9 ± 3.6 years] with a median follow-up of 2.8 years (interquartile range [IQR] 1.7-4.3). A total of 23 features were clustered in three classes according to their prevalence in UC: six class-1 features [0% prevalence in UC], 12 class-2 features [< 5% prevalence], and five class-3 features [5-10% prevalence]. According to the algorithm, the disease should be classified as UC if no features exist in any of the classes. When at least one feature exists, different combinations classify the disease into atypical UC, IBDU or CD. The algorithm differentiated UC from CD and IBDU with 80% sensitivity (95% confidence interval [CI] 71-88%) and 84% specificity [77-89%], and CD from IBDU and UC with 78% sensitivity [67-87%] and 94% specificity [89-97%].

CONCLUSIONS

The validated PIBD-classes algorithm can adequately classify children with IBD into small bowel CD, colonic CD, IBDU, atypical UC, and UC.

摘要

背景

修订后的波尔图标准确定了儿童炎症性肠病的亚型：溃疡性结肠炎[UC]、非典型UC、未分类的炎症性肠病[IBDU]和克罗恩病[CD]。其他人提出了克罗恩结肠炎的另一种亚分类。在延续波尔图标准的基础上，我们旨在推导并验证名为“PIBD分类”的标准，以规范不同炎症性肠病亚型的分类。

方法

这是一项来自ESPGHAN波尔图小组下属23个中心的多中心回顾性纵向研究。采用假设驱动的判断方法和数学分类与回归树[CART]建模来创建诊断算法。由于小肠炎症很容易被识别为CD，我们在此主要关注结肠炎的表型。

结果

总共纳入了749例炎症性肠病儿童：236例[32%]克罗恩结肠炎、272例[36%]UC和241例[32%]IBDU[年龄10.9±3.6岁]，中位随访时间为2.8年（四分位间距[IQR]1.7 - 4.3）。根据其在UC中的患病率，共23个特征被聚类为三类：6个1类特征[在UC中的患病率为0%]、12个2类特征[患病率<5%]和5个3类特征[患病率5 - 10%]。根据该算法，如果任何一类中都不存在特征，则疾病应分类为UC。当至少存在一个特征时，不同的组合将疾病分类为非典型UC、IBDU或CD。该算法区分UC与CD和IBDU的灵敏度为80%（95%置信区间[CI]71 - 88%），特异度为84%[77 - 89%]，区分CD与IBDU和UC的灵敏度为78%[67 - 87%]，特异度为94%[89 - 97%]。