Abstract
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).
Aims
In continuation of the Porto criteria, we aimed to further derive and validate criteria for standardizing the diagnosis of the IBD subtypes with an emphasis on IBDU, the least well defined subtype.
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 jejunal and ileal inflammation is easily recognized as CD, we focused here on colitis phenotype.
Results
749 IBD children were enrolled- 236 (32%) Crohn’s colitis (CD), 272 (36%) ulcerative colitis (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 set of 23 features were clustered in 3 classes according to their frequency 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, UC should be diagnosed if no features exist in the three classes. Different combinations of the features classify atypical UC, IBDU and CD. The algorithm differentiated UC from CD and IBDU with 80% sensitivity (95% CI (71–88)) and 84% specificity (95% CI (77–89)), and CD from IBDU and UC with 78% sensitivity (95% CI (67–87)) and 94% specificity (95% CI (89–97)).
Conclusions
The validated algorithm can adequately classify children with IBD into CD, UC and IBDU.
Funding Agencies
None