Abstract Background Perianal Crohn's disease (CD) is characterised by fistulae and/or abscesses in the anal region and leads to significant morbidity. We aimed to estimate the incidence and describe the disease course of perianal disease in paediatric-onset CD patients (<18 years at CD diagnosis). Methods In a nationwide registry-based cohort study, we identified all incident patients with paediatric-onset CD in Denmark from 1980 to 2018. We followed the patients until emigration, death, or December 31, 2022, and recorded the cumulative incidence of perianal disease based on data from the National Patient Registry. Using perianal disease as an exposure, we further assessed the risks of major abdominal surgeries, colorectal and anal cancer, and mortality. We used multivariate Cox regression analysis with biological age as the timescale to estimate hazard ratios adjusted (aHR) for sex, family income, and calendar year of CD diagnosis. Results We identified 2,356 paediatric-onset CD patients, of whom 769 (32.6%) were diagnosed with perianal CD. Total follow-up time was 38,584 person-years. The cohort is described in detail in Table 1. The cumulative incidence of perianal CD was 14.0%, 21.1%, and 28.1% after 1, 5, and 10 years after CD diagnosis, respectively. The incidence density during the study period was 29 (95% confidence interval [CI]: 26.8–30.9) per 1,000 person-years, corresponding to 45.2% after 30 years of follow-up. Among the 769 patients with perianal CD, 460 (59.8%) had perianal abscesses, 399 (51.9%) perianal fistula, and 627 (81.5%) patients underwent perianal surgery. Of CD patients with perianal disease, 308 (40.1%) required a stoma compared to 147 (9.3%) of patients without, corresponding to an aHR of 2.8 (95% CI: 2.3-3.4). When comparing CD patients with perianal disease to those without, the aHRs for major abdominal surgery, cancer, and mortality were 1.5 (95%CI: 1.3–1.8), 0.8 (95% CI: 0.2–2.7), and 1.5 (95%CI: 0.9–2.7), respectively. Of the 68 patients that died, 35 (2.2/1,000 person-years) had perianal disease, whereas 33 (1.4/1,000 person-years) had not. Further risk analysis is presented in Figure 1. Conclusion In this nationwide cohort of patients with paediatric-onset CD (<18 years at CD diagnosis), 29 patients per 1,000 person-years developed perianal disease between 1980-2022. Patients with perianal CD were more likely to require major abdominal surgery, including stoma surgery, and had a higher risk of mortality compared to patients without perianal CD. Our study underlines the severity of perianal disease in paediatric-onset CD.
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