by Kimberly Allen, RN
Primary biliary cirrhosis is a chronic condition in which the bile ducts in your liver are slowly destroyed. So far this disease has only been found in adults and is much more common in women than men. In fact 90% of the cases are women. Primary biliary cirrhosis or PBC is usually diagnosed in adults between 30 and 60 years of age. Although is is an uncommon disease it is not rare.
The bile ducts are basically the plumbing system for the liver. They are lined with specialized cells called biliary epithelial cells. The biliary duct system starts with very small diameter ducts that connect to larger diameter ducts that branch out into larger diameter ducts that is frequently referred to as the biliary tree. The large duct on the right as well as the one on the left then connect to a single even larger duct known as the common bile duct. the common bile duct runs outside the liver to the small intestine. Bile is the fluid produced by the cells in the liver called hepatocytes. It is crucial for the digestion and absorption of fat and other waste products like biliruben. When PBC starts the inflammation begins in the very small bile ducts. As the small bile ducts are destroyed the normal flow of bile into your small intestine is blocked reducing the flow of bile. This is called cholestasis. As the inflammation spreads it destroys more of the bile ducts and eventually spreads to the hepatocytes. As the hepatocytes are destroyed scar tissue forms and spreads through the areas that have been destroyed. The progressive inflammation along with the scarring and toxicity of the bile that is trapped in the hepatocytes combined together leads to the development of cirrhosis.
While the exact cause of PBC is not clear research indicates there could be several causes including an autoimmune malfunction or there may be a genetic alteration that predisposes a person to PBC. There have also been indications that certain infections can lead to PBC. Most experts believe PBC is an autoimmune disease that is triggered by some type of fleeting infection. However, there are some that point to certain characteristics of PBC that are uncharacteristic for autoimmune disorders like the fact that all other autoimmune disorders occur in children as well as adults and PBC has never been diagnosed in children, it is so far an adults only disease.
many people with PBC have no symptoms until there is significant damage to their liver. However, there are also many people that do experience symptoms and in the early stages the most commonly reported symptoms are chronic fatigue and itchy skin that can range from mild to severe. Once PBC has progressed and there is extensive damage to your liver most people will experience symptoms associated with the inability of the liver to function properly including jaundice and edema in your feet and legs as well as your abdomen. Some people will also develop fatty deposits on the skin, especially around the eyes.
So far there is no cure for PBC so treatment is focused on preventing as much liver damage as possible, relieving symptoms and preventing complications. Your doctor may recommend ursodeoxycholic acid or UDCA which is a bile acid that helps the bile to move through your liver and is generally considered to be the first line of treatment. While UDCA doesn’t cure PBC if started early in the disease it may help prolong life. The other alternative that your doctor may recommend when other treatments no longer control the disease and your liver begins to fail is a liver transplant. However, transplant surgery comes with it’s own set of potential complications and frequently PBC will recur in the transplanted liver.
It is also important for you to make lifestyle adjustments to improve liver function including quitting smoking if you’re a smoker, and limiting or eliminating alcohol consumption as well as maintaining a healthy weight. It’s also important to eat a low sodium diet and exercise regularly. And, because your liver isn’t functioning properly it’s very important to talk to your doctor before starting any new medications including over the counter medicines, especially any that contain acetaminophen as well as any dietary supplements.
Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at firstname.lastname@example.org.