Primary Biliary Cholangitis (PBC)

The cause is not clear but is probably an autoimmune reaction Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers an autoimmune disorder is not known. Symptoms vary depending... read more (in which the immune system attacks the body’s own tissues). Primary biliary cholangitis often occurs in people with autoimmune disorders, such as rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more , scleroderma Systemic Sclerosis Systemic sclerosis is a rare, chronic autoimmune connective tissue disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities... read more , Sjögren syndrome Sjögren Syndrome Sjögren syndrome is a common autoimmune connective tissue disorder and is characterized by excessive dryness of the eyes, mouth, and other mucous membranes. White blood cells can infiltrate... read more , or autoimmune thyroiditis Hashimoto Thyroiditis Hashimoto thyroiditis is chronic, autoimmune inflammation of the thyroid gland. Hashimoto thyroiditis results when antibodies in the body attacks the cells of the thyroid gland—an autoimmune... read more .

An autoimmune cause is also thought possible because more than 95% of people with PBC have certain abnormal antibodies in their blood. These antibodies attack mitochondria (tiny structures that produce energy in cells). However, these antibodies are not involved in the destruction of the bile ducts. Other immune cells attack the bile ducts. What triggers this attack is unknown, but it may be exposure to a virus or a toxic substance.

Usually, PBC starts very gradually. About half of people have no symptoms at first.

The first symptoms often include

Other problems may not occur until months or years later:

Eventually, any of the symptoms and complications of cirrhosis Symptoms Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more can develop. Fats, including fat-soluble vitamins (A, D, E, and K), are often poorly absorbed. Poor absorption of vitamin D results in osteoporosis Osteoporosis Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more , and poor absorption of vitamin K contributes to easy bruising and bleeding. If the body cannot absorb fats, stools may be light-colored, soft, bulky, oily-looking, and unusually foul-smelling (called steatorrhea).

The liver and spleen may enlarge. But as scarring progresses, the liver shrinks.

A doctor may suspect this disorder in middle-aged women who have typical symptoms such as fatigue and itchiness. However, in many people, the disorder is discovered long before symptoms appear because results of routine blood tests to evaluate the liver Liver Blood Tests Liver tests are blood tests that represent a noninvasive way to screen for the presence of liver disease (for example, viral hepatitis in donated blood) and to measure the severity and progress... read more are abnormal.

During the physical examination, the doctor may feel an enlarged, firm liver (in about 25% of people) or an enlarged spleen Enlarged Spleen An enlarged spleen is not a disease in itself but the result of an underlying disorder. Many disorders can make the spleen enlarge. Many disorders, including infections, anemias, and cancers... read more (in about 15%).

If PBC is suspected, doctors do liver tests, imaging tests, and blood tests to check for antibodies to mitochondria.

Imaging tests Imaging Tests of the Liver and Gallbladder Imaging tests of the liver, gallbladder, and biliary tract include ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography... read more are done to check for abnormalities or blockages in bile ducts outside the liver. These tests include magnetic resonance imaging (MRI) of the bile duct system (called magnetic resonance cholangiopancreatography, or MRCP) and often ultrasonography. If results of these tests are not clear, endoscopic retrograde cholangiopancreatography (ERCP) may be done. For this procedure, an x-ray is taken after a substance that can be seen on x-rays (contrast agent) is injected through a viewing tube (endoscope) inserted through the mouth into the bile ducts. Finding no blockages outside the liver means that the liver is the site of the problem and thus supports the diagnosis of PBC.

A liver biopsy Biopsy of the Liver Doctors can obtain a sample of liver tissue during exploratory surgery, but more often they obtain a sample by inserting a hollow needle through the person's skin and into the liver. This type... read more (removal of a tissue sample for examination under a microscope) is usually done to confirm the diagnosis. Biopsy also helps doctors determine how advanced the disorder is (the stage).

Primary biliary cholangitis usually progresses slowly, although how fast it progresses varies greatly. Symptoms may not appear for 2 years or for up to 10 to 15 years. Some people become very ill in 3 to 5 years. Once symptoms develop, life expectancy is about 10 years. Certain characteristics suggest that the disorder will progress quickly:

Once jaundice Jaundice in Adults In jaundice, the skin and whites of the eyes look yellow. Jaundice occurs when there is too much bilirubin (a yellow pigment) in the blood—a condition called hyperbilirubinemia. (See also Overview... read more develops, death may occur within months.

There is no known cure. Treatment includes

No alcohol should be consumed. Drugs that may damage the liver are stopped.

Cholestyramine may control itchiness, as may rifampin, naltrexone (an opioid), sertraline, or ursodeoxycholic acid plus ultraviolet light.

Ursodeoxycholic acid, particularly if used before the disorder is advanced, reduces liver damage, prolongs life, and delays the need for liver transplantation. Obeticholic acid has been proven to improve liver-related blood tests in many people with PBC for whom ursodeoxycholic acid alone is not effective. It should not be used in people with advanced cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more , because it can worse liver function.

Supplements of calcium and vitamin D are needed to help prevent osteoporosis Osteoporosis Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more or slow its progression. Weight-bearing exercises, bisphosphonates, or raloxifene may also help prevent or slow osteoporosis. Vitamin A, D, E, and K supplements may be needed to correct vitamin deficiencies. Vitamins A, D, and E can be taken by mouth. Vitamin K is given by mouth or injection.

Liver transplantation Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more is the best treatment when the disorder is advanced. It can prolong life. After transplantation, PBC recurs in some people, but it rarely becomes severe.

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