Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear. Bile is a liquid produced in the liver that travels through the bile ducts to the gallbladder and then the small intestine, where it helps digest fats and fat-soluble vitamins A, D, E, and K. When the bile ducts become damaged from chronic inflammation, bile builds up in the liver, injuring liver tissue.

Injured liver tissue from chronic inflammation and the buildup of bile leads to cirrhosis, a condition in which the liver slowly deteriorates and malfunctions. Scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. Scarring also impairs the liver’s ability to:

• Control infections.
• Remove bacteria and toxins from the blood.
• Process nutrients, hormones, and drugs.
• Make proteins that regulate blood clotting.
• Produce bile to help absorb fats – including cholesterol – and fat-soluble vitamins.
• Effectively replace its own cells when they become damaged.

Primary biliary cirrhosis develops over time and may ultimately cause the liver to stop working completely. Most people are diagnosed early, before the disease progresses. Early treatment delays, but does not stop, the eventual onset of cirrhosis and liver failure. When a person has end-stage (complete, or almost complete phase of a terminal disease) liver disease, a liver transplant is necessary for survival.

Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men.

Diagnosis of Primary Biliary Cirrhosis

The first indication of primary biliary cirrhosis may occur when results of routine blood tests to check liver function are abnormal. The doctor will then order one or more tests to confirm the disease:

• Anti-Mitochondrial Antibody (AMA) Blood Test
  The presence of AMA is detected in 90 percent of people with primary biliary cirrhosis.
• Alkaline Phosphatase Blood Test
  Primary biliary cirrhosis is likely if two blood tests performed at least 6 months apart reveal alkaline phosphatase – a liver enzyme – to be abnormally high.
• Liver Biopsy
  A liver biopsy can confirm the diagnosis but is not always necessary. A biopsy may help determine the extent of liver damage. The biopsy is performed with a needle inserted between the ribs or into a vein in the neck. Precautions are taken to minimize discomfort. A tiny sample of liver tissue is examined with a microscope for scarring or other signs of cirrhosis. Sometimes a cause of liver damage other than cirrhosis is found during biopsy.

The doctor may also order the following tests:

• Cholesterol Blood Test
  People with primary biliary cirrhosis may have abnormally high levels of cholesterol in the blood. However, these high cholesterol levels are usually less harmful to people with primary biliary cirrhosis than to those without the disease.
• Abdominal Ultrasound
  An ultrasound shows whether the liver and bile ducts are inflamed. A handheld device, which a technician glides over the abdomen, sends sound waves toward the abdomen. The sound waves bounce off the liver and other organs, and their echoes create a picture of the liver and biliary system on a video monitor.

Complications of Primary Biliary Cirrhosis

Some people develop one or more complications of primary biliary cirrhosis when the disease progresses to cirrhosis.

Osteoporosis
The disease may cause bones to become fragile and more likely to break. Osteoporosis can also result from steroid use as a treatment of primary biliary cirrhosis.

Maldigestion
When a person with primary biliary cirrhosis has jaundice, the small intestine cannot easily absorb fats and fat-soluble vitamins. Maldigestion may result in diarrhea and fatty stools and can lead to weight loss in the late stages of the disease.

Portal Hypertension
Normally, blood from the intestines and spleen is carried to the liver through the portal vein. But primary biliary cirrhosis may cause inflammation in the portal tract, leading to increased pressure in the portal vein. This condition is called portal hypertension.

Esophageal Varices and Gastropathy
When portal hypertension occurs, it may cause enlarged blood vessels in the esophagus, called varices, or in the stomach, called gastropathy, or both. Enlarged blood vessels are more likely to burst due to thin walls and increased pressure. If they burst, serious bleeding can occur in the esophagus or upper stomach, requiring immediate medical attention.

Splenomegaly
When portal hypertension occurs, the spleen frequently enlarges and sequesters or holds white blood cells and platelets, reducing the numbers of these cells in the blood. A low platelet count in the blood may be the first evidence that a patient has developed cirrhosis.

Edema and Ascites
When the liver damage progresses to an advanced stage, fluid collects in the legs, called edema, and in the abdomen, called ascites. Ascites can lead to bacterial peritonitis, a serious infection.

Bruising and Bleeding
When the liver slows or stops producing the proteins needed for blood clotting, a person will bruise or bleed easily.

Sensitivity to Medications
Cirrhosis slows the liver’s ability to filter medications from the blood. When this occurs, medications act longer than expected and build up in the body. This causes a person to be more sensitive to medications and their side effects.

Hepatic Encephalopathy
A failing liver cannot remove toxins from the blood, and they eventually accumulate in the brain. The buildup of toxins in the brain – called hepatic encephalopathy – can decrease mental function and cause coma. Signs of decreased mental function include:

• Confusion
• Personality changes
• Memory loss
• Trouble concentrating
• A change in sleep habits

Insulin Resistance and Type 2 Diabetes
Cirrhosis causes resistance to insulin – a hormone produced by the pancreas that enables the body to use glucose as energy. With insulin resistance, the body’s muscle, fat, and liver cells do not use insulin properly. The pancreas tries to keep up with the demand for insulin by producing more, but excess glucose builds up in the bloodstream causing type 2 diabetes.

Liver Cancer
Hepatocellular carcinoma is a type of liver cancer that can occur in patients with cirrhosis. Hepatocellular carcinoma has a high mortality rate, but several treatment options are available.

Other Problems
Cirrhosis can cause immune system dysfunction, leading to the risk of infection. Cirrhosis can also cause kidney and lung failure, known as hepatorenal and hepatopulmonary syndromes.

For More Information About Primary Biliary Cirrhosis

American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY 10038
Phone: 1-800-GO-LIVER (465-4837)
1-888-HEPUSA (443-7872), or 212-668-1000
Fax: 212-483-8179
Email: info@liverfoundation.org
Internet: www.liverfoundation.org

United Network for Organ Sharing
P.O. Box 2484
Richmond, VA 23218
Phone: 1-888-894-6361 or 804-782-4800
Fax: 804-782-4817
Internet: www.unos.org

National Digestive Diseases Information Clearinghouse (NDDIC)
2 Information Way
Bethesda, MD 20892-3570
Phone: 1-800-891-5389
TTY: 1-866-569-1162
Fax: 703-738-4929
Email: nddic@info.niddk.nih.gov
Internet: www.digestive.niddk.nih.gov

Hope through Research
The National Institute of Diabetes and Digestive and Kidney Diseases’ Division of Digestive Diseases and Nutrition supports basic and clinical research into liver diseases, including primary biliary cirrhosis, and liver transplantation. Researchers are studying:

• The mechanisms of liver injury and regeneration.
• Ways to improve outcomes of liver transplantation.
• How autoimmune liver diseases develop in the body.

Participants in clinical trials can play a more active role in their own health care, gain access to new research treatments before they are widely available, and help others by contributing to medical research. For information about current studies, visit www.ClinicalTrials.gov.

Source: digestive.niddk.nih.gov – December 2008