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P202 Outcomes of a large cohort of children with IBDU compared to other IBD subtypes and treatment options- a longitudinal report from the Porto pediatric IBD group of ESPGHAN

M. Aloi1, L. Birimberg-Schwartz2, I. Hojsak3, S. Buderus4, A. Paerregaard5, J. Bronsky6, J. Fell7, S. Koletzko8, G. Veereman9, R. Shaoul10, E. Miele11, D. Turner2, R. Russell*12

1Sapienza University of Rome, Pediatrics, Rome, Italy, 2Shaare Zedek Medical Center, Department of Pediatrics, Jerusalem, Israel, 3Clinical Hospital Center Sestre Milosrdnice, Department of Pediatrics, Zabreb, Croatia, 4Marien Hospital, Department of Pediatrics, Bonn, Germany, 5Hvidovre Hospital, Department of Pediatrics, Hvidovre, Denmark, 6Charles University Prague, Department of Pediatrics, Prague, Czech Republic, 7Chelsea and Westminster Hospital, Paediatric Gastroenterology, London, United Kingdom, 8Helmholtz Zentrum Munchen, Department of Pediatrics, Munich, Germany, 9Uz Brussel, Department of Pediatrics, Bruxelles, Belgium, 10Rambam Medical Center, Department of Pediatrics, Haifa, Israel, 11Federico II University, Department of Pediatrics, Naples, Italy, 12York Hill Hospital, Department of Pediatrics, Glasgow, United Kingdom

Background

Inflammatory bowel disease unclassified (IBDU) is the rarest subtype of IBD and as such treatment options for patients are based mainly on extrapolation from ulcerative colitis (UC) and Crohn's disease (CD) studies. We aimed to look at treatment choices for IBDU compared to other patients with colonic IBD and to compare patient outcomes at 3 years.

Methods

This was a multicentre retrospective longitudinal study including 23 centres affiliated with the Porto IBD-working group of ESPGHAN. Data on 797 children with colonic IBD were collected on a standard proforma at diagnosis and follow up with strict diagnostic (Porto) criteria [mean age 10.5 ± 3.9, 46% females]: 250 with CD, 287 with UC and 260 with IBDU. Disease severity was assessed by Physician Global Assessment (PGA).

Results

IBDU treatment significantly differed from that of UC for lower use of corticosteroids (CS) [154 (59%) vs. 204 (71%), p=0.004] but higher use of exclusive enteral nutrition (EEN) [26 (10%) vs. 2 (0.6%), p<0.0001]. Compared to CD, IBDU patients received less EEN and immunomodulators [26 (10%) vs. 93 (37%), p<0.0001 and 67 (26%) vs. 129 (52%) p<0.0001, respectively] but more aminosalicylates [228 (88%) vs. 159 (64%), p<0.0001]. At 3-year follow-up, 135 (69%) IBDU patients had a remission or a mild disease compared to 100 with CD (46%, p<0.0001), and 174 with UC (64%, p=0.3). CD patients were more commonly treated with biologics than those with IBDU and UC [82 (34%) vs. 24 (12%) IBDU and 47 (17%) UC; p<0.0001, vs IBDU and UC]. Four patients with IBDU (1%) underwent surgery during follow up, vs. 22 (5%) with UC (p=0.009) and 20 (5%) with CD (p=0.008 vs. IBDU), while no significant differences for time for surgery were reported among the three groups. A significantly higher proportion of IBDU patients treated with CS at the diagnosis required biologics and cyclosporine at follow-up, compared to those treated with other therapies (p=0.04 and p=0.05, respectively).

Conclusion

Children with IBDU receive less 5ASA/steroids than UC but more than CD yet receive less EEN and immunomodulators than CD but more than UC. The need for biologics and surgery at follow up is lower in IBDU than CD. CS use at the diagnosis in IBDU patients is related to worse clinical outcomes. Despite these differences a mild disease course in IBDU patients at follow-up is common.