What is it?

The liver is an organ that is housed in the right upper side of your abdomen, mostly behind the ribs. It is about the size of a football and has many functions. It stores energy and nutrients, makes proteins, breaks down and filters substances, processes old red blood cells, and kills germs that enter the body through the intestines. The liver is also responsible for making bile. Bile is a fluid that is made up of water and other particles that are produced, stored in the gallbladder, and eventually arrives in the small intestine. Bile is important for the digestion and absorption of fats and vitamins that are fat-soluble. Also, many waste products, such as bilirubin, a product that remains when old red blood cells are broken down, are excreted in bile and then eliminated from the body in a bowel movement.

In primary biliary cirrhosis (PBC), the immune system attacks the small bile ducts, eventually leading to the destruction of the ducts. This leads to trouble in allowing bile to flow from the liver to the small intestine. The continued restriction of the flow of bile leads to inflammation, scarring, tissue injury, cirrhosis, and ultimately liver failure.

Who gets it?

  • It occurs more often in women, 95% of the patients are women
  • Onset is usually between 30-65 years old and is rare before age 30 unlike other autoimmune disorders


Why does it happen?

The exact cause is unknown, but it seems to be related to factors in a person’s genes and the environment such as toxins or infection with bacteria or a virus. Regardless of the cause something causes, the body’s immune system to attack the small bile ducts.

What are the symptoms?

You may have no symptoms at all. 50-60% of patients feel completely normal. Others have:

  • Fatigue
  • Itchiness. It is often worse at night, worse under clothes, especially if tight or coarse material, with dry skin, or hot, humid weather.
  • Skin changes. 25-50% of patients have increased pigment of their skin. This is not jaundice or yellowing of the skin, but actually due to extra melanin being deposited
  • Joint symptoms
  • Right upper abdominal pain
  • Bone disease
  • Memory or concentration problems


How is it diagnosed?

This disorder will be considered if you have symptoms or a certain substance in your blood, alkaline phosphatase, is elevated. Other things can also cause this substance to be elevated, so things like drugs and alcohol use will be ruled out. Pictures may be taken of the liver to make sure that there are no blockages. Specific proteins that the immune system makes will be tested for: antimitochondrial, antinuclear, and antismooth muscle antibodies. Also, a tissue sample, a biopsy of the liver, may be obtained.

How is it treated?

There are two goals of treatment, to relieve symptoms or complications, and to slow down the destruction of the bile ducts.

Malabsorption can be addressed by decreasing the intake of fat because normal levels of bile are not present to help us break down fat. Vitamins that dissolve in fat (vitamins A, D, E, and K) will be replaced if they are low. Rarely, levels of cholesterol will build, and xanthomas, deposits of cholesterol in the skin, will form. These are treated by blood draws that decrease the level of cholesterol. Sometimes, people will develop dry eyes, mouth, and vagina. Artificial tears, saliva substitutes, medications, and moisturizers can be used. If the disease progresses to where the liver is failing, this is treated similarly to other forms of cirrhosis and liver failure.

The only medication that is used to slow down the attack on the bile ducts is ursodeoxycholic acid (ursodiol, UDCA). It is not exactly clear how this works, but it delays the progression to end-stage liver disease, but not all people respond. Other treatments are currently under investigation. The American Association for the Study of Liver Diseases suggests routine follow up with this disease including:

  • Monitoring liver tests every 3-6 months
  • Checking thyroid function every year
  • Checking the density of bones and evaluating bone health every 2-4 years
  • Monitoring of vitamin A, D, and K levels every year if bilirubin is elevated over 2
  • Other recommendations are also available if there is liver damage or failure


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