Development and validation of diagnostic criteria for IBD subtypes with an emphasis on IBD-Unclassified in children: a multicenter study from the Pediatric IBD Porto group of ESPGHAN

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

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Abstract / Description of output

Background: The revised Porto criteria identify subtypes of pediatric inflammatory bowel diseases: ulcerative colitis (UC), atypical UC, Inflammatory Bowel Disease Unclassified (IBDU), and Crohn's disease (CD). In continuation of the Porto criteria, we aimed to derive and validate criteria for standardizing the classification of the IBD subtypes.

Methods: This was a multicenter retrospective longitudinal study from 23 centers affiliated with the Porto-group of ESPGHAN. Both a hypothesis-driven judgmental approach and mathematical CART modeling were utilized for creating a diagnostic algorithm. Since small bowel inflammation is easily recognized as CD, we focused here primarily on the phenotype of colitis.

Results: 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 (IQR 1.7-4.3). A total of 23 features were clustered in 3 classes according to their prevalence in UC: 6 class-1 (0% prevalence in UC), 12 class-2 (<5% prevalence) and 5 class-3 (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 exist, different combinations classify the disease into atypical UC, IBDU and CD. The algorithm differentiated UC from CD and IBDU with 80% sensitivity (95%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 algorithm can adequately classify children with IBD into small bowel CD, Colonic CD, IBDU, atypical UC and UC.

Original languageEnglish
JournalJournal of Crohn's and Colitis
DOIs
Publication statusPublished - 18 Apr 2017

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