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Prognosis of pediatric-onset inflammatory bowel disease associated with primary sclerosing cholangitis: A
Marie-Laura Godet1, Hélène Sarter2,3, Frédéric Gottrand1,2
1Gastroenterology, Hepatology, and Nutrition Unit, Department of Pediatrics, CHU Lille, Lille, France.
Insights
Pediatric inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC) carries a worse prognosis due to increased cancer and mortality risks. The IBD course itself is not significantly altered by PSC, but the co-occurrence elevates long-term health risks.
Area of Science:
- Gastroenterology
- Pediatric Gastroenterology
- Hepatology
Background:
- Pediatric-onset inflammatory bowel disease (IBD) prognosis can be complex.
- Primary sclerosing cholangitis (PSC) is a chronic liver disease often associated with IBD.
- The impact of PSC on the long-term outcomes of pediatric IBD is not fully understood.
Purpose of the Study:
- To evaluate if the prognosis of pediatric-onset IBD is affected by its co-occurrence with PSC.
- To compare cancer risk, mortality, medical treatment, and bowel resection rates in pediatric IBD patients with and without PSC.
Main Methods:
- A retrospective, population-based study utilized data from the EPIMAD Registry.
- Pediatric IBD patients diagnosed with PSC (IBD-PSC) were compared to matched pediatric IBD patients without PSC (matched-IBD).
- Propensity score matching ensured comparability based on sex, age, year, and IBD diagnosis location; PSC confirmation involved imaging and/or histology.
Main Results:
- The study included 24 IBD-PSC cases and 96 matched IBD controls with a median follow-up of 6.4 years.
- No significant differences were found in the use of immunosuppressants, corticosteroids, or anti-TNF therapy at 5 years between groups.
- IBD-PSC cases exhibited a 28-fold increased cancer risk and a 13-fold increased mortality risk compared to the general population; no such increase was seen in IBD-only patients.
Conclusions:
- Pediatric-onset IBD associated with PSC has a significantly worse prognosis than IBD without PSC.
- This poorer prognosis is primarily driven by substantially elevated rates of cancer and mortality.
- The underlying course of IBD appears unaffected by PSC, but the co-morbidity introduces severe systemic risks.
Objectives:
To assess whether the prognosis of pediatric-onset inflammatory bowel disease (IBD) is influenced by its association with primary sclerosing cholangitis (PSC) considering medical treatment, bowel resection, risk of cancer, and mortality.
Methods:
A retrospective population-based study was conducted using data from the EPIMAD Registry, one of the most extensive prospective population-based IBD studies globally. Cases (IBD-PSC) were compared with matched controls (matched-IBD). Inclusion criteria were age ≤17 years at IBD diagnosis and follow-up ≥2 years. PSC was confirmed by magnetic resonance cholangiopancreatography and/or histology. Each case was matched to four controls by propensity score (i.e., sex, age, year, and location of IBD at diagnosis).
Results:
We included 24 cases and 96 controls. Median duration of follow-up was 6.4 years (interquartile range = 3.1-14.3). No significant difference was observed between the two groups in terms of exposure to treatment at 5 years (immunosuppressants, corticosteroids, or antitumor necrosis factor). In IBD-PSC, cancers were 28 times more frequent (standardized incidence ratio = 27.9; 95% confidence interval [CI], 7.0-111.7, p = 0.002), and death was 13 times more frequent (standardized mortality ratio = 13.3; 95% CI, 3.3-53.4, p = 0.010) than in the general population. No increased risk of cancer or mortality was observed in patients with IBD but without PSC compared to the general population.
Conclusion:
Although the course of IBD is not different, the prognosis of pediatric-onset IBD associated with PSC is significantly worse than that of pediatric-onset IBD without PSC because of increased cancer and mortality rates.
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