Clinical presentation depends upon the aetiology, and may involve either chronic symptoms or an acute episode. Pruritus, fatigue and jaundice, with steatorrhoea and deranged LFTs (raised alkaline phosphatase and serum bilirubin in PBC, raised alanine aminotransferase [ALT] and aspartate minotransferase [AST] in alcoholic disease) are generally present by the later stages. This is followed by the symptoms of portal hypertension (see below), which is a poor prognostic feature associated with late-stage cirrhosis.
The process may be reversed in alcoholics who stop drinking. However the prognosis of any cirrhotic condition is extremely poor if malignancy is present. In severe cases, the management revolves around trying to treat the symptoms of portal hypertension rather than the disease itself.
Liver transplant is now an established and highly successful treatment option for PBC when the symptoms can no longer be controlled with drugs.
It is also an option for alcoholic cirrhosis, although there is currently a significant incidence of posttransplant return to alcoholism.
HCC = hepatocellular carcinoma.