Primary (autoimmune) sclerosing cholangitis in patient with inflammatory bowel disease
Authors:
Ľ. Podracká; A. Šuláková
Authors‘ workplace:
Klinika dětského lékařství, Fakultní nemocnice Ostrava-Poruba
přednosta doc. MUDr. M. Hladík, Ph. D.
Published in:
Čes-slov Pediat 2015; 70 (6): 329-332.
Category:
Case Report
Overview
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease caused by inflammation and fibrosis of the intra and//or extrahepatic bile ducts and can ultimately progress to biliary cirrhosis and hepatic failure. Incidence and prevalence of PSC in children is not exactly know, the 0.23 per 100,000 patients, which is about 5 times less than in adults. The disease is in 85% of causes associated with the inflammatory bowel disease (IBD). In the case that the primary sclerosing cholangitis is associated with an autoimmune presentation, it is necessary to think of overlapping syndrome by AIH/PSC (autoimmune hepatitis), it means sclerosing autoimmune cholangitis (ASC). Major diagnostic method is cholangiography (magnetic resonance cholangiopancreatography) and liver histology.
We present a case of a 13-year-old boy examinated for hepatopathy. The elevation of the ALP, GMT, abnormal imunoglobulin lewels and the presence of anti-neutrophil cytoplasmic antibodies (ANCA) have led to suspicions on the PSC. Liver biopsy confirmed the simultaneous presence of autoimmune inflammation and led to the diagnosis of overlapping syndrome (autoimmune sclerosing cholangitis). During kolonoscopy was found inflammatory of the cecum and Bauhin´s valve and during magnetic resonance enterography (MRE) was found inflammatory od the aboral part of terminal ileum that was histologically confirmed.
Key words:
primary sclerosing cholangitis, overlapping syndrome, inflammatory bowel disease, cholestasis
Sources
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Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2015 Issue 6
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