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P152. Rising incidence and increasing severity of very early onset IBD in Ireland

R. Wylde1, A. Carey2,3, B. Bourke2,3,4, A. Broderick3, S. Quinn3, M. Hamzawi3, K. Gleeson3, S. Hussey2,3,4, 1Leiden University, Leiden University Medical Centre, Leiden, Netherlands, 2National Childrens Research Centre, NCRC, Dublin, Ireland, 3Our Lady's Childrens Hospital Crumlin, Dept. of Gastroenterology, Hepatology and Nutrition, Dublin, Ireland, 4University College Dublin (UCD), School of Medicine and Medical Science, Dublin, Ireland

Background

The literature describing the epidemiology and outcomes of very early onset IBD (VEO-IBD) is limited. This study examined the epidemiology, phenotype and clinical outcomes of a national cohort of Irish children with VEO-IBD from 2000 to 2012.

Methods

A retrospective review of all cases of VEO-IBD (those diagnosed <10 years of age) attending the National Centre for Paediatric Gastroenterology (NCPG) from January 2000 to December 2012 was undertaken. Patient demographics, diagnostic work-up, initial and subsequent treatment, and long term clinical and surgical outcomes at 1, 2, 5 and/or maximum clinical follow up were recorded. Cases were phenotyped according to the Paris classification and clinical activity was determined using PGA, PCDAI and PUCAI scores. Poisson regression analysis was used to calculate incidence trends.

Results

One hundred and fifty-eight children (50% male) with VEO-IBD were identified; 78 (49%) had Crohn Disease (CD), 63 (40%) had Ulcerative Colitis (UC) and 17 (11%) had IBD-unclassified (IBD-U). Median age of onset was 7.5 years (IQR 3.42 years). The incidence of VEO-IBD ranged from 0.8 to 3.3/100,000/year. The mean rate of increase was 4% per year (p = 0.06; CI 0%, 8%). The rate of increase was significant in males (p = 0.04) compared to females (p = 0.281). The incidence of UC increased strikingly from 0.6 to 1.6/100,000 (CI 0.025, 0.164) at an average annual rate of 9%. Pancolonic UC increased 4-fold from 0.6 to 2.3/100,000/year (p = 0.005). Using PUCAI scores, severe disease activity at presentation increased significantly from 0% to 36% (p = 0.002). The incidence of CD increased at a slower rate of 0.02/100,000/year (CI −0.41, 0.079). Significantly more males (44%) than females (18%) had both upper and lower disease activity (p = 0.03). 76% of patients with CD had inflammatory disease behaviour while 24% had stricturing or penetrating disease. At total of 31% of children presented with perianal disease. At one year follow up (n 148), 64% were in clinical remission, with 88 (59%) in steroid-free remission. Twenty-five (17%) children commenced immunomodulators within 1 year of diagnosis while 6 (4%) underwent surgery. At maximum follow up (n 154; median 60 months), 24 (16%) children had commenced biologics, 43 (28%) immunomodulators and 20 (13%) had undergone surgery (12 UC; 8 CD).

Conclusion

This is the first national population-based study describing the epidemiology and outcomes of VEO-IBD phenotypes. Boys had more extensive disease than girls. Substantial and sustained increases in the incidence and severity of UC in particular were observed. Prospective longitudinal studies are required to fully elucidate the factors underlying the aetiology and outcome of VEO-IBD.