Colitis-associated sclerosing cholangitis in children: a single centre experience

J Crohns Colitis. 2013 Nov;7(10):e414-8. doi: 10.1016/j.crohns.2013.01.016. Epub 2013 Feb 26.

Abstract

Objective: Sclerosing cholangitis (SC) is an important immune-mediated extra-intestinal manifestation of inflammatory bowel disease (IBD), primarily affecting patients with ulcerative colitis (UC). The reported prevalence of SC in adults and children with UC is low at between 2 and 7%. We present findings from a hepatological work-up in children with inflammatory colitis and elevated liver function tests (LFT) from a tertiary paediatric gastroenterology unit.

Design: This study is designed as a retrospective review of the medical records of 17 children and adolescents with inflammatory colitis and abnormal LFTs who presented to our IBD service between April 2004 and April 2012.

Results: Over the eight year period a total of 52 patients were diagnosed with inflammatory colitis (ulcerative colitis and unclassified colitis). Seventeen of the 52 patients had abnormal liver function tests and underwent liver biopsy and cholangiography. All 17 patients (32.6%) were diagnosed with hepato-biliary disease.

Conclusion: This is one of the largest reported series of children with inflammatory colitis and associated hepato-biliary disease. The data from this patient group indicate that the prevalence of IBD-associated hepato-biliary disease in children with abnormal LFTs is much higher than previously reported. As the diagnosis of IBD-associated hepato-biliary disease affects patient management, we recommend liver biopsy and cholangiography in all children with inflammatory colitis and abnormal liver function tests.

Keywords: Auto-immune liver disease; Hepato-biliary disease;; Inflammatory bowel disease;; Sclerosing cholangitis;; Ulcerative colitis;.

MeSH terms

  • Adolescent
  • Alanine Transaminase / blood
  • Anti-Inflammatory Agents / therapeutic use
  • Biopsy
  • Child
  • Cholagogues and Choleretics / therapeutic use
  • Cholangiopancreatography, Magnetic Resonance
  • Cholangitis, Sclerosing / complications*
  • Cholangitis, Sclerosing / diagnosis*
  • Cholangitis, Sclerosing / drug therapy
  • Colitis, Ulcerative / complications*
  • Colitis, Ulcerative / diagnosis*
  • Colitis, Ulcerative / pathology
  • Female
  • Humans
  • Intestinal Mucosa / pathology
  • Liver / pathology*
  • Liver Function Tests
  • Male
  • Prednisolone / therapeutic use
  • Retrospective Studies
  • Ursodeoxycholic Acid / therapeutic use
  • gamma-Glutamyltransferase / blood

Substances

  • Anti-Inflammatory Agents
  • Cholagogues and Choleretics
  • Ursodeoxycholic Acid
  • Prednisolone
  • gamma-Glutamyltransferase
  • Alanine Transaminase