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Long-term outcome of pediatric-onset Crohn's disease: A population-based cohort study
Mathurin Fumery1, Benjamin Pariente2, Helene Sarter3
1Gastroenterology Unit, Amiens University and Hospital, Université de Picardie Jules Verne, Amiens, France.
Insights
Pediatric-onset Crohn's disease (CD) often leads to severe outcomes, with most patients developing complicated disease behavior over time. Early ileal or ileocolonic and perianal lesions are key risk factors for disease progression.
Area of Science:
- Pediatric Gastroenterology
- Inflammatory Bowel Disease Research
- Clinical Epidemiology
Background:
- Pediatric-onset Crohn's disease (CD) is often more severe than adult-onset disease.
- Understanding long-term outcomes and risk factors for disease progression is crucial.
Purpose of the Study:
- To describe the long-term outcomes of a large cohort of pediatric-onset CD patients.
- To identify risk factors associated with complicated disease behavior in this population.
Main Methods:
- Retrospective analysis of the EPIMAD registry (1988-2004).
- Inclusion of pediatric CD patients (<17 years) with at least two years of follow-up.
- Multivariate Cox regression to identify risk factors for complicated behavior.
Main Results:
- 535 patients with a median follow-up of 11.1 years.
- 83% had ileocolonic disease; 42% had L4 disease; 16% had perianal disease.
- 58% developed complicated behavior; ileal, ileocolonic, and perianal lesions at diagnosis were significant risk factors (p<0.05).
- 43% underwent intestinal resection; overall mortality 0.93%; cancer SIR 3.3 (p=0.01).
Conclusions:
- The majority of pediatric-onset CD patients develop complicated disease behavior.
- Ileal, ileocolonic, and perianal lesions at diagnosis are associated with a higher risk of complicated disease.
- Long-term outcomes include significant rates of surgery and a slightly increased risk of cancer.
Background:
Pediatric-onset Crohn's disease (CD) may represent a more severe form of disease. The aim of this study was to describe long-term outcome and identify associated risk factors of complicated behavior in a large population-based pediatric-onset CD cohort.
Patients And Methods:
Cases included all patients recorded in the EPIMAD registry diagnosed with definite or probable CD between January 1988 and December 2004, under the age of 17 years at the time of diagnosis, with at least two years of follow-up.
Results:
Five hundred and thirty-five patients were included. Median follow-up was 11.1 years [IQR, 7.3-15.0]. At the end of follow-up, 8% (n = 44) of patients had pure ileal disease (L1), 8% (n = 44) had pure colonic disease (L2), and 83% (n = 439) had ileocolonic disease (L3). L4 disease and perianal disease were observed in 42% (n = 227) and 16% (n = 85) of patients, respectively. At the end of follow-up, 58% (n = 308) of patients presented complicated disease behavior (B2, 39% and B3, 19%), and 42% (n = 163) of patients with inflammatory behavior at diagnosis had evolved to complicated behavior. During follow-up, 86% of patients (n = 466) received at least one course of corticosteroids, 67% (n = 357) of patients had been exposed to immunosuppressants and 35% (n = 187) of patients received at least one anti-TNF agent. Forty-three percent (n = 230) of patients underwent at least one intestinal resection. The overall mortality rate was 0.93% and the SMR was 1.6 [0.5-3.8] (p = 0.20). Five cancers were reported with a crude cancer incidence rate of 1.1% and an SIR of 3.3 [1.2-7.0] (p = 0.01). In a multivariate Cox model, ileal (HR, 1.87 [1.09-3.21], p = 0.022) or ileocolonic (HR, 1.54 [1.01-2.34], p = 0.042) and perianal lesions at diagnosis (HR, 1.81 [1.13- 2.89], p = 0.013) were significantly associated with complicated behavior.
Conclusion:
About 80% of patients with pediatric-onset CD presented extensive ileocolonic disease during follow-up. The majority of patients evolved to complicated behavior. Surgery, cancer and mortality were observed in 43%, 0.9% and 0.9% of patients, respectively.
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