IBD and hepatobiliary disease (primary sclerosing cholangitis)

Hepatobiliary involvement is seen in 10% to 15% of children and adolescents who have inflammatory bowel disease and is among the most common extraintestinal manifestation associated with these disorders. The nature of the relationship between inflammatory bowel disease and hepatobiliary disease remains unclear. Although the course of the liver disease usually is mild, it is more severe in patients who have ulcerative colitis than Crohn disease. The findings associated with these disorders include fatty infiltration, primary sclerosing cholangitis, pericholangitis, chronic hepatitis, cirrhosis, and acalculous cholecystitis.

Although biochemical or histologic abnormalities such as fatty infiltration and pericholangitis of the liver are found in approximately 50% of pediatric patients who have ulcerative colitis, only 10% to 14% of these children become symptomatic, with jaundice and pain being uncommon. Children who have hepatobiliary involvement are at risk for malignancy (biliary tract carcinoma) and may require liver transplantation. Some studies suggest that the incidence of severe liver damage is higher among patients who have pancolitis than among those in whom disease involvement is  limited to the descending colon.

Primary sclerosing cholangitis develops in 3% of children who have inflammatory bowel disease. Chronic ulcerative colitis more often is  associated with sclerosing cholangitis than is Crohn disease, representing 74% of the cases. It may be present during the initial evaluation for inflammatory disease in up to 20% of patients; only 50% of children who have sclerosing cholangitis have inflammatory bowel disease. Sclerosing cholangitis is characterized by fibrosis and inflammation of the intrahepatic and extrahepatic bile ducts, leading to bile duct obliteration and hepatic failure. The etiology is unknown, but it probably is mediated immunologically. Presenting  symptoms include abdominal pain, fatigue, weight loss, jaundice, and pruritus, as described for the boy in the vignette. Patients who have primary sclerosing cholangitis have elevated serum aminotransferase and gamma glutamyl transferase and direct bilirubin concentrations. Antinuclear and anti-smooth muscle antibodies are present in most patients. Endoscopic retrograde cholangiopancreatography (ERCP) is the diagnostic procedure of choice. The pathognomonic findings at ERCP include diffusely distributed multifocal strictures alternating with dilated or normal segments. Treatment of primary sclerosing cholangitis includes immunosuppression, ursodeoxycholic acid, antibiotics, and liver transplantation, although none has proved palliative or curative.

Although cholelithiasis, hepatic abscess, and hepatitis B infection can occur in patients who have inflammatory bowel disease, none of these disorders occurs more commonly in patients who have either ulcerative colitis or Crohn disease.

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