Incidence and Natural History of Very Early-Onset Inflammatory Bowel Disease in Scotland: National and Regional Cohort Studies.
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BACKGROUND: Very early-onset inflammatory bowel disease (VEOIBD) (diagnosed under 6 years of age) has attracted considerable attention in recent years, with monogenic forms providing insight into immune dysregulation. However, the majority of VEOIBD patients have a classical polygenic phenotype and a similar disease course to older children. METHODS: The study sought to assess the incidence trends of VEOIBD in Scotland from 1981 to 2014 and to describe the natural history, phenotype, and treatment burden of VEOIBD in a regional cohort from South-East Scotland (1997-2021). RESULTS: Nationally, 128 (8.1%) of 1567 incident pediatric inflammatory bowel disease (IBD) patients (<16 years of age at diagnosis) were VEOIBD; 10 (8%) of 128 were diagnosed <2 years of age. The incidence of VEOIBD rose from 0.64 per 100 000 per year (1981-1985) to 2.73 per 100 000 per year (2011-2014) (P = .002), an incidence rate ratio of 4.3 (95% confidence interval, 2.4-8.3). The average annual percentage change was 3.9% (95% confidence interval, 2.1%-5.7%) (P < .05). Crohn's disease was the most common subtype (63%); the median age at diagnosis was 4.4 years. The regional cohort (n = 46) had a median follow-up of 11.9 years. Crohn's disease cases presented with isolated colonic involvement in 47%, with pancolitis present in 64% of ulcerative colitis cases. Treatment exposures included immunosuppression (76%), corticosteroids (67%), and anti-tumor necrosis factor therapies (50%). A total of 11% were on no IBD-specific therapy at last follow-up, 11% required surgery and no patient had a diagnosis of monogenic IBD, cancer, or death. CONCLUSIONS: Scotland has a high incidence of VEOIBD, with monogenic IBD exceptionally rare. Although VEOIBD disease burden is high, treatment outcomes are broadly similar to those diagnosed later in childhood based on previous studies.