Hepatology

Primary Biliary Cirrhosis

Primary biliary cirrhosis - also known as primary biliary cholangitis - is a type of liver disease that causes damage to the bile ducts in the liver. Over time, the disease fully destroys the bile ducts, which are responsible for carrying bile from the liver to the small intestine.

When the bile ducts function normally, bile that's made in the liver is transported to the gallbladder and then to the small intestine to assist in the digestive process and help rid the body of cholesterol and toxins. When the bile ducts are destroyed, bile builds up and causes liver damage and scarring, leading to a variety of symptoms and serious health complications.

Primary biliary cirrhosis can be diagnosed in either gender and at any age, but it is most common among women between the ages of 30 and 60.

Primary Biliary Cirrhosis Symptoms

Primary biliary cirrhosis is an autoimmune disease, meaning that the body's immune system is rebelling and mistakenly attacking healthy tissue - in this case, the bile ducts.

People with primary biliary cirrhosis may not experience symptoms at first or after they're initially diagnosed. However, over time, people with primary biliary cirrhosis may develop a wide range of symptoms affecting the entire body.

Symptoms may include:

• Abdominal pain
• Dry eyes and mouth
• Fatigue
• Fluid buildup in the abdomen
• High cholesterol
• Hypothyroidism
• Itchy skin
• Jaundice, or yellowing of the skin and eyes
• Musculoskeletal pain affecting the bones, muscles, or joints
• Skin hyperpigmentation
• Swollen feet or ankles
• Weight loss  

Complications of primary biliary cirrhosis include gallstones or bile duct stones; osteoporosis; portal hypertension, which is high blood pressure in the vein to the liver; and spleen enlargement. 

It is important to seek medical treatment when symptoms or complications first emerge, since in many cases, treatment with medication can slow liver damage. The condition generally progresses slowly, so early intervention can improve outcomes. 

Primary Biliary Cirrhosis Treatment

If your physician believes you have primary biliary cirrhosis, he or she will take a thorough personal and family medical history, perform a physical exam and order other tests, including blood tests, imaging, and/or a liver biopsy. 

If you're diagnosed with the condition, a number of treatment options - including medications, supplementation with fat-soluble vitamins, or surgery - are available to alleviate symptoms and slow or delay the disease progress. In cases of liver failure, a liver transplant may be necessary. 

To prevent further liver damage, your physician will likely also recommend lifestyle changes, including quitting smoking, not drinking alcohol, eating a well-balanced diet, and maintaining a healthy weight.