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Primary Biliary Cirrhosis (cont.)

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Hepatorenal syndrome

Patients with advanced liver disease and portal hypertension can sometimes develop the hepatorenal syndrome. This syndrome is a serious problem with the functioning of the kidneys without actual physical damage to the kidneys themselves. The hepatorenal syndrome is defined by progressive failure of the kidneys to clear substances from the blood and produce adequate volumes of urine even though some other kidney functions, such as retention of salt, are maintained. If liver function improves or a healthy liver is transplanted into a patient with hepatorenal syndrome, the kidneys often begin to work normally. This restoration of kidney function indicates that liver failure is associated with an inability of the liver to either produce or detoxify substances that affect kidney function.

Hepatopulmonary syndrome

Rarely, some patients with advanced cirrhosis can develop the hepatopulmonary syndrome. These patients can experience difficulty with breathing because certain hormones released in advanced cirrhosis cause abnormal functioning of the lungs. The basic lung problem in the hepatopulmonary syndrome is that the blood flowing through the small vessels in the lungs does not come in sufficient contact with the alveoli (air pockets) of the lungs. Therefore, the blood cannot pick up enough oxygen from the air that is breathed and the patient experiences difficulty breathing.

Liver cancer (hepatocellular carcinoma)

Patients with PBC that develop cirrhosis have an increased risk of developing a primary cancer of the liver cells (hepatocytes) called liver cancer (hepatocellular carcinoma). Primary refers to the fact that the tumor originates in the liver. A secondary tumor originates elsewhere in the body and can spread (metastasize) to the liver.

Cirrhosis due to any cause increases the risk of liver cancer. Therefore, the development of a primary liver cancer in an individual with PBC is not unexpected. However, the risk of hepatocellular carcinoma in PBC appears to be lower than the risk in cirrhosis caused by some other liver diseases, such as chronic viral hepatitis. A recent report indicated that hepatocellular carcinoma might be more common in men than women with PBC. Indeed, this one series of 273 patients with advanced PBC found hepatocellular carcinoma in 20% of the men compared to only 4.1% of the women. The way hepatocellular cancer develops in PBC, however, is not understood.

The most common symptoms and signs of primary liver cancer are abdominal pain and swelling, an enlarged liver, weight loss, and fever. In addition, these liver tumors can produce and release a number of substances, including ones that cause an increase in red blood cells (erythrocytosis), low blood sugar (hypoglycemia), and high blood calcium (hypercalcemia).

The most useful diagnostic tests for hepatocellular carcinoma are a blood test called an alpha-fetoprotein and an imaging study of the liver (either a CT Scan or an MRI with intravenous dye/contrast). The best screening tests for early detection of hepatocellular carcinoma in patients with cirrhosis are serial alpha-fetoprotein levels and ultrasound examinations of the liver every 6 to 12 months. It is important to note that about 40% of hepatocellular cancers do not produce elevated levels of alpha-fetoprotein.



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