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P211. IBD-Unclassified in childhood and adolescence; a complicated diagnosis

D.A. Winter1, J.C. Escher1, E. Porto2, IBD Working Group of ESPGHAN, 1Erasmus MC-Sophia Children's Hospital, Pediatric Gastroenterology, Rotterdam, Netherlands, 2European Society for Paediatric Gastroenterology, Hepatology and Nutrition, Porto, Portugal

Background

IBD-unclassified (IBD-U) is diagnosed in about 10% of pediatric-or adolescent onset IBD patients (PIBD). According to the original Porto criteria [1], a provisional diagnosis of IBD-U is warranted only when a final classification cannot be made despite complete diagnostic work-up. Typically, disease activity in IBD-U is limited to the colon but without typical features of Crohn's disease (CD) or ulcerative colitis (UC). Data on demographic characteristics, diagnostic work-up and disease phenotype of IBD-U patients aged 0–18 years were analysed. In addition, diagnostic work-up was analysed according to size of including center.

Methods

The IBD working group of ESPGHAN initiated the EUROKIDS database May 2004 to prospectively monitor diagnostic work-up of newly diagnosed PIBD patients. To reach uniformity in diagnostic work-up of PIBD patients consensus-based criteria were created in 2005. These original Porto criteria advise diagnostic work-up by esophagogastroduodenoscopy (EGD) and ileocolonoscopy including histology of multiple biopsies from all segments, as well as small bowel imaging in all suspected IBD patients. We defined complete diagnostic work-up as complete EGD and colonoscopy (including histology), as well as visualization of the terminal ileum through endoscopy or radiology. Fifty-two centers from 21 different countries participated. For reasons of uniformity, patients diagnosed and registered from May 2005 until November 2013 were used for analysis.

Results

IBD-U was attributed to 267/3463 patients (7.7%), in 52% (139/267) complete diagnostic work-up had been performed. Large centers performed complete diagnostic work-up in 71% of IBD-U patients, significantly more often than small (37.5%) and medium centers (52%) (p = 0.001). One in eight patients (33/267) had features more compatible with CD such as radiologic disease of the small bowel (6/267), granuloma (6/267) or perianal disease (14/267).

Conclusion

Almost half (48%) has been inadvertently diagnosed with IBD-U as they did not fulfill criteria of a complete diagnostic work-up. In addition, 12% had features compatible with CD. Using strict criteria, a diagnosis of IBD-U was justified in only 127 patients (3.7%). Correct phenotype classification is essential for treatment decisions and prognosis and can only be done when diagnostic work-up is complete. Completeness of diagnostic work-up seems dependent on center size. Adherence to a complete diagnostic work-up, as well as the updated Porto criteria (in preparation) that will define more clearly the classification of PIBD, will likely lead to a decrease in patients with IBD-U. Results from the EUROKIDS database reinforce the importance of a complete diagnostic work-up in new PIBD patients.

1. IBD Working Group of ESPGHAN, (2005), Inflammatory bowel disease in children and adolescents: recommendations for diagnosis--the Porto criteria, Journal of Pediatric Gastroenterology and Nutrition, 1–7, http://www.ncbi.nlm.nih.gov/pubmed/15990620