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[Association between Crohn's disease and primary sclerosing cholangitis in a 10 year old girl]
Ana Muñoz Lozón1, Cristina Iglesias Blázquez2, Cristina Menéndez Arias2
1Servicio de Pediatría del Complejo Asistencial, León, España. amunozlozon@gmail.com.
Insights
A rare case of Crohn's disease and primary sclerosing cholangitis in a child highlights diagnostic challenges. This association, typically seen in adult males, requires advanced imaging for confirmation and has a poor prognosis.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Inflammatory Bowel Disease Research
Background:
- Inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC) are chronic inflammatory conditions.
- The co-occurrence of Crohn's disease (CD) and PSC is infrequent, particularly in pediatric populations.
- PSC is a rare, chronic liver disease characterized by inflammation and fibrosis of the bile ducts.
Observation:
- A 10-year-old girl presented with symptoms suggestive of IBD, including diarrhea, abdominal pain, weight loss, and fever.
- Initial investigations and endoscopy confirmed Crohn's disease.
- Development of cholestasis and persistent hypergammaglobulinemia led to further investigation.
Findings:
- Magnetic resonance cholangiography and liver biopsy confirmed concomitant primary sclerosing cholangitis.
- The patient's presentation and diagnosis underscore the clinical variability of CD and PSC association.
- Diagnostic confirmation relied on advanced imaging techniques like magnetic resonance cholangiography.
Implications:
- This case emphasizes the importance of considering rare comorbidities in pediatric IBD patients, even with atypical demographics.
- Early and accurate diagnosis of both CD and PSC is crucial for management, despite limited treatment options.
- The poor prognosis associated with this rare comorbidity necessitates further research into therapeutic strategies and disease progression.
Abstract:
A 10 year old girl with diarrhea, abdominal pain, weight loss and fever of one month and a half of evolution. Analytical and sonographic findings raised the possibility of inflammatory bowel disease. Endoscopy and histology showed findings consistent with Crohn's disease. Treatment was initiated with mesalazine and exclusive enteral nutrition. Later corticosteroid treatment, immunosuppressive drugs and ursodeoxycholic acid were added due to cholestasis and persistent hypergammaglobulinemia. Magnetic resonance cholangiography and liver biopsy confirmed the diagnosis of concomitant primary sclerosing cholangitis. The association between Crohn's disease and primary sclerosing cholangitis is rare, predominantly in males between 20 and 40 years old and it presents a great clinical variability. The confirmation of the diagnosis requires magnetic resonance cholangiography or endoscopic retrograde cholangiopancreatography. The prognosis is poor and there is no treatment to slow the progression of the disease.
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