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.
References:
Classen M, Gotze H, Richter HJ, Bender S. Primary sclerosing
cholangitis in children. J Pediatr Gastroenterol Nutr. 1987;6:197-202
Desmet VJ, Geboes K. Liver lesions in inflammatory bowel disease. J
Pathol. 1987;151:247-255
Hyams J, Markowitz J, Treem W, et al. Characteristics of hepatic
abnormalities in children with inflammatory bowel disease. Inflamm
Bowel Dis. 1995;1:27-33
Kane W, Miller K, Sharp HL. Inflammatory bowel disease presenting as
liver disease during childhood. J Pediatr. 1980;97:775-778
Vierling JM. Hepatobiliary complications of ulcerative colitis and
Crohn disease. In: Zakim D, Boyer TD, eds. Hepatology: A Textbook of
Liver Disease. 3rd ed. Philadelphia, Pa: WB Saunders Co; 1990:
1126-1158