Primary Biliary Cirrhosis
Clinical Primary Biliary Cirrhosis
Of unknown pathogenesis, primary biliary cirrhosis is probably neither autoimmune nor infectious in origin, although it is associated with autoimmune hepatitis. A strong association exists with antimitochondrial antibodies (AMAs), yet some patients are AMA negative and antinuclear antibody positive; the latter is also called autoimmune cholangitis (or cholangiopathy) rather than primary biliary cirrhosis.
Whether patients with so-called autoimmune cholangitis (those with an absence of antimitochondrial antibodies) should be consigned to a separate disease entity or considered to have a variant of primary biliary cirrhosis is conjecture (autoimmune cholangitis .
Few differences are evident in clinical and serologic features in AMA-negative and AMA-positive primary biliary cirrhosis patients.
A relationship exists between primary biliary cirrhosis and Sjogren’s syndrome. In fact, Sjogren’s syndrome is one of the most frequent extrahepatic associations of primary biliary cirrhosis, and some investigators consider that primary biliary cirrhosis is a secondary form of Sjogren’s syndrome. Celiac sprue appears to be more common among patients with primary biliary cirrhosis than the general population. A rare patient also has chronic autoimmune gastritis or idiopathic thrombocytopenic purpura. No association is believed to exist with inflammatory bowel disease, which distinguishes this condition from sclerosing cholangitis. Pregnancy probably has no effect on
ciation between primary biliary cirrhosis and bronchial asthma has been reported. Severe hypoxemia has developed. Patients with primary biliary cirrhosis are believed not to be at increased risk for developing hepatocellular carcinoma. Nevertheless, hepatocellular carcinoma and even a cholangiocarcinoma does occur occasionally. The majority of patients are middle-aged women. Typically an early prodromal stage with mild elevation of liver function tests leads to cholestasis, although some patients are symptomatic even prior to cholestasis. Sacral insufficiency fractures have developed in women with primary biliary cirrhosis.
CT Abdomen with contrast cirrhosis
Imaging differentiation between primary biliary cirrhosis and sclerosing cholangitis is generally straightforward. A cholangiographic “pruned-tree” appearance gradually becomes evident .
CT Abdomen with contrast indications A cirrhotic liver in a 24-year-old man with cystic fibrosis and hepatic failure. The liver has a nodular outline. Splenomegaly and vascular congestion (arrowheads) are secondary to portal hypertension. (Courtesy of Douglas S. Katz, M.D., Winthrop University Hospital, Mineola, New York.)