Primary Biliary Cirrhosis (cont.)
John M. Vierling, MD, FACP
John M. Vierling M.D. is Professor of Medicine and Surgery at the Baylor College of Medicine in Houston, Texas, where he also serves as Director of Baylor Liver Health and Chief of Hepatology. In addition, he is the Director of Advanced Liver Therapies, a center devoted to clinical research in hepatobiliary diseases at St. Luke's Episcopal Hospital. Dr. Vierling is board certified in internal medicine and gastroenterology and a Fellow of the American College of Physicians.
Leslie J. Schoenfield, MD, PhD
Dr. Schoenfield served as associate professor of medicine and consultant in gastroenterology on the faculty of the Mayo Clinic for seven years. He became a professor of medicine in residence at UCLA from 1972 to 1999 (now emeritus). He was the director of gastroenterology at Cedars-Sinai Medical Center in Los Angeles for 25 years, where he received the chief resident's teaching award, the president's award, and the pioneer of medicine award.
In this Article
- What is PBC?
- What is the scope of the problem?
- What is the cause of PBC?
- What are the symptoms and physical findings in PBC?
- What manifestations are specifically due to PBC itself?
- What are the manifestations of the complications of cirrhosis in PBC?
- What are the manifestations of diseases associated with PBC?
- What are risk factors for PBC?
- How is PBC diagnosed?
- What is the role of blood tests?
- What is the role of testing for antimitochondrial antibodies?
- What is the role of imaging tests?
- What is the role of liver biopsy?
- What are the criteria for a definitive diagnosis of PBC
- What is the course of natural progression in PBC?
- What are the sequential clinical phases of PBC?
- What is the role of mathematical models in predicting the outcome (prognosis) in PBC?
- What about pregnancy in PBC?
- Find a local Gastroenterologist in your town
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.
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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