Incidence and Natural History of Very Early-Onset Inflammatory Bowel Disease in Scotland: National and Regional Cohort Studies

TY - JOUR

T1 - Incidence and Natural History of Very Early-Onset Inflammatory Bowel Disease in Scotland

T2 - National and Regional Cohort Studies

AU - Henderson, Paul M.

AU - Gerasimidis, Konstantinos

AU - Russell, Richard K.

AU - Chalmers, Iain

AU - Hansen, Richard

AU - Wilson, David C.

N1 - © The Author(s) 2025. Published by Oxford University Press on behalf of Crohn’s & Colitis Foundation. All rights reserved.

PY - 2026/2

Y1 - 2026/2

N2 - 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.

AB - 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.

KW - epidemiology

KW - Crohn’s disease

KW - ulcerative colitis

KW - preschool child

KW - cost of illness

U2 - 10.1093/ibd/izaf229

DO - 10.1093/ibd/izaf229

M3 - Article

C2 - 41124317

SN - 1078-0998

VL - 32

SP - 261

EP - 270

JO - Inflammatory Bowel Diseases

JF - Inflammatory Bowel Diseases

IS - 2

M1 - izaf229

ER -