Evolution of the phenotypic characteristics of pediatric Inflammatory Bowel Disease using the Paris classification

The recent Paris classification modifies the Montreal classification in recognition of phenotypic features specific to childhood-onset disease, more specifically, incorporating categories to distinguish patients presenting prior to 10 years of age along with the ability to capture growth impairment. Disease phenotype, particularly disease behaviour, has the potential to evolve over time. Recent European paediatric studies have suggested a greater tendency for CD and UC to extend in childhood, in comparison with adult patients. to examine the evolution of disease location and behaviour over time, in young North American paediatric IBD patients, using Paris criteria for phenotypic classification. From January 2000 to December 2004, 264 children aged less than 15 years of age were newly diagnosed with IBD. 142 (56.8%) CD; 107 (40.5%) UC; 15 (5.7%) IBD-U. The median age at diagnosis for CD was 11.9 years (IQR 9.7-13.2), for UC: 11.1 years (IQR 7.6-13.0) and for IBD-U: 12.2 years (IQR 5.8-13.4). The median follow-up time was 5.6 years (IQR 3.9-7 years). Patients were classified by the new Paris criteria as to maximal IBD location and behaviour at diagnosis and at latest follow-up. Treatments were recorded. In comparison to older (A1b) children, the youngest (Ala) with CD had less L1 disease (2 of 39 vs 27 of 103, p = 0.005 OR 0.15 (95% CI 0.03-0.67)) and consequently more colonic disease (L2 + L3 36 of 39 vs 72 of 103, p = 0.004, OR 5.17 (95% CI 1.48 -18.05)). Among the youngest (Ala) children with initially colonic CD (L2), extension to the ileum occurred in 5 of 14 (35.7%) patients. UC was already extensive (E3 or E4) at diagnosis in 78% of all children. By 2 and 5 yrs, respectively, medical treatment included immunomodulators in 55.6% and 71.2 % of all 142 CD patients and in 32.6 % and 39.3% of all 107 UC patients. By 2 and 5 years, anti-TNF-alpha therapy was initiated in 14.1% and 36.4% of all CD patients and in 6.5% and 10.1% of all UC patients. The cumulative incidence of intestinal resection in children with CD was 7.1% and 16.6%, respectively, at 2 and 5 years. Colectomy was performed in 7.5% and 13.7% of UC patients by 2 and 5 years, respectively. The Paris separation of age category into A1a (< 10yrs) and A1b (>10 years) highlights the rarity of isolated ileal CD in children presenting prior to 10 years of age, and the propensity of their colonic CD to subsequently extend. CD location in children ≥ 10 years remained stable and mirrors that observed in adults, whereas childhood onset UC is more extensive at the outset. Perianal fistulizing disease became more common, and CD behaviour progressed from inflammatory to stricturing or penetrating over time in children of all ages.

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