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P085. Primary sclerosing cholangitis in the course of inflammatory bowel disease in children


U. Chlebowczyk1, S. Wiecek1, M. Kajor2, H. Wos1

1Silesian Medical University, Department of Paediatrics, Katowice, Poland; 2Silesian Medical University, Department of Pathology, Katowice, Poland



Background: Hepatic manifestations are one of the most common parenteral symptoms of inflammatory bowel disease (IBD). Diseases of the liver and bile ducts may have the pathomechanism that is common with IBD; such diseases include: primary sclerosing cholangitis (PSC), small duct PSC, and PSC/AIH overlap syndrome.

The aim of this study was to evaluate the incidence of PSC and AIH/PSC incourse of inflammatory bowel disease in children, and to assess the clinical course of inflammatory bowel disease in children with and without coexisting cholangitis.

Methods: The study involved 172 children: 92 with ulcerative colitis and 80 with Crohn's disease, at the ages of 4–18 years. IBD was diagnosed on the basis of the Porto Criteria. In all the study children disease activity was evaluated according to PUCAI and PCDAI scales; the following immunoassays were performed: p‑ANCA, IgA ASCA, and IgG ASCA. PSC was diagnosed on the basis of results of laboratory liver function tests, ultrasound imaging, cholangio-MR and histopathological examination of liver biopsies. With regard to a very low incidence of hepatic complications in children with Crohn's disease (1 AIH and 1 PSC/AIH), the statistical analysis was only performed in children with ulcerative colitis. The statistical analysis was based on the Mann-Whitney U test and Statistica 9 software.

Results: In course of UC in examined children we found PSC in 12 (13%) – in 3 cases it was overlap syndrome PSC/AIH. Statistically, PSC was significantly more frequent in boys (p < 00.5). In the group of children with PSC/UC, p‑ANCA antibodies were twice more frequent (p < 00.2). The analysis of the location of inflammatory lesions in the large intestine demonstrated that in the group of children with PSC/UC, inflammatory lesions in the rectum were absent in half of these patients (p < 0.01) Moreover, insignificantly more frequent involvement of the whole large intestine was observed in this group, when compared with children affected by UC.

Conclusions:

  1. Bile duct diseases in the course of inflammatory bowel disease occur more frequently in children than in adult patients.
  2. Differences in the clinical course of inflammatory bowel disease with or without accompanying cholangitis are related to sex and extent of inflammatory lesions in the large intestine.