Primary biliary cirrhosis is a chronic condition that causes bile duct inflammation. Prolonged inflammation of the bile ducts causes them to become damaged and not function properly.
In the event of chronic inflammation in the bile ducts and the liver, eventually harmful scar tissue develops. An excessive amount of scar tissue in the liver is a serious condition called cirrhosis. Cirrhosis prevents the liver from functioning and is potentially fatal. Liver failure may result from this excessive scarring, making a liver transplant necessary for survival.
The cause of primary biliary cirrhosis is currently unknown. It is believed to be an automimmune condition, but this is not certain. Autoimmune disorders cause the body's immune system to attack bodily tissue. The body's immune system attacks the bile ducts of the liver in cases of primary biliary cirrhosis.
Individuals with autoimmune conditions tend to be at a higher risk for developing primary biliary cirrhosis. As autoimmune conditions often have a genetic link, it is believed that primary biliary cirrhosis may also have a genetic cause. Individuals with a predisposition to autoimmune disorders may be more likely to develop primary biliary cirrhosis after an infection or exposure to certain toxins.
Many individuals are diagnosed with primary biliary cirrhosis prior to the manifestation of physical symptoms. When symptoms are present they may include:
Malaise ( a generalized sense of feeling unwell)
Pruritus (itchy skin)
Jaundice (the yellowing of the skin and the whites of eyes)
Dry eyes and/or mouth
Often primary biliary cirrhosis is discovered when abnormal results for a liver function test are present. Other tests that may be used to diagnose primary biliary cirrhosis include:
Anti-mitochondrial antibody (AMA). AMA is found in the blood of nearly every individual with primary biliary cirrhosis.
Alkaline phosphate blood test. Elevated levels of alkaline phosphate in the blood stream found twice in six months could indicate primary biliary cirrhosis.
Liver biopsy. In some cases, a liver biopsy may be necessary to help confirm a primary biliary cirrhosis diagnosis. Additionally, a biopsy can help determine the extent of liver damage resulting from the condition.
Diagnostic imagining tests. Tests such as an Ultrasound, may be used to investigate inflammation in the liver or the bile ducts.
Cholesterol level test. Because many individuals with primary biliary cirrhosis have abnormally high levels of cholesterol in the blood, a cholesterol test may be used to help diagnose primary biliary cirrhosis.
The initial goal of primary biliary cirrhosis treatment is usually centered around managing symptoms such as the discomfort of pruritis. Currently there is no treatment to reverse damaged caused to the bile ducts or to the liver.
The medication flavoxate has been approved for the treatment of primary biliary cirrhosis. This medication cannot cure primary biliary cirrhosis but may prove effective in slowing down the progression of damage caused by the condition. Other ways to slow the progression of the disease are to maintain a nutritious diet, avoid excessive sodium intake, avoid alcohol, drugs and medications as well as exposure to other toxins.
In some cases, a liver transplant may be necessary to treat primary biliary cirrhosis. A liver transplant is the only treatment that can cure the condition.
Primary biliary cirrhosis typically develops in individuals between the ages of 40 and 60. The condition affects more women than men.