Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) and is a very unpleasant chronic disease leading to serious liver damage. The disease has certain similarities with the disease known as primary sclerosing cholangitis (PSC).


The disease is caused by an autoimmune process that leads to inflammation of small bile ducts within the liver. The reason is unclear; probably it is a genetically-influenced condition with some participation of certain outer factors. The disease typically occurs in women and it is much rare in men. It is frequently associated with rheumatoid arthritis and Sjögren's syndrome.


The bile ducts are damage and this causes disruption of proper bile flow out of the liver. As the bile accumulates, the patient begins to suffer from itchy skin (due to deposits of bile salts) and gradually developing jaundice (deposits of bilirubin). Decreased amount of bile that is excreted into the intestine is reason of impaired absorption of nutrients from the digestive tract. The stool is greasy and smells unpleasantly. As time passes, liver damages progresses to cirrhosis with portal hypertension and all its complications (ascites, esophageal varices, hepatic encephalopathy and many others). The condition may end as fatal acute liver failure. A patient with primary biliary cirrhosis has increased risk of liver cancer.


In addition to the clinical signs, it is important to exclude other common reasons of liver damage, especially infectious hepatitis and chronic alcoholism. In a blood smear we find elevated liver tests and increased level of bilirubin. Special serologic examination of the blood may confirm a presence of certain autoantibodies in serum (autoantibodies known as ANA and AMA). Abdominal ultrasound demonstrated signs of liver cirrhosis and upper GI endoscopy may find esophageal varices. It is very important to perform a liver biopsy to get a tissue sample for histological examination under a microscope.


The treatment is difficult. To a certain extent we are able to improve the flow of bile, administer some hepatoprotective drugs and treat the itching. The disease itself is treated by various immunosuppressive drugs, but it often progress to state, where it is necessary to evaluate the possibility of liver transplant. The overall prognosis without transplantation is not very good and the average survival period is only few years after the diagnosis. This is a difference compared to PSC, which has significantly better prognosis.


Total alcohol abstinence is necessary. In this case, the alcohol is not the reason of liver damage, but it may significantly accelerate the condition and speed up the liver failure.


Jiri Stefanek, MD  Author of texts: Jiri Stefanek, MD
 Sources: basic text sources