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Primary Biliary Cholangitis (PBC)

(Primary Biliary Cirrhosis)

By

Jesse M. Civan

, MD, Thomas Jefferson University Hospital

Last full review/revision Dec 2019| Content last modified Dec 2019
Click here for the Professional Version

Primary biliary cholangitis (PBC) is inflammation with progressive scarring of the bile ducts in the liver. Eventually, the ducts are blocked, the liver becomes scarred, and cirrhosis and liver failure develop.

Primary biliary cholangitis (PBC) is most common among women aged 35 to 70, although it can occur in men and women of any age. It tends to occur in families.

The liver produces bile, a greenish yellow, thick, sticky fluid that aids digestion. Bile also helps eliminate certain waste products (mainly bilirubin and excess cholesterol) and by-products of drugs from the body. Bile ducts are small tubes that carry bile from the liver to the gallbladder and then to the small intestine. Primary biliary cholangitis affects only the small bile ducts inside the liver and the liver cells near these bile ducts. Another inflammatory bile duct disorder, primary sclerosing cholangitis Primary Sclerosing Cholangitis Primary sclerosing cholangitis is inflammation with progressive scarring and narrowing of the bile ducts in and outside the liver. Eventually, the ducts become blocked and then obliterated.... read more , affects bile ducts inside and outside the liver.

Primary biliary cholangitis begins with inflammation of the bile ducts. The inflammation blocks the flow of bile out of the liver. Thus, bile remains in the liver cells, causing inflammation. If damage from the inflammation is mild, the liver commonly repairs itself by making new liver cells and attaching them to the web of connective tissue (internal structure) that is left when liver cells die. As inflammation spreads, a latticework of scar tissue (fibrosis Fibrosis of the Liver Fibrosis is the formation of an abnormally large amount of scar tissue in the liver. It occurs when the liver attempts to repair and replace damaged cells. Many conditions can damage the liver... read more ) develops throughout the liver. The scar tissue performs no function, and it can distort the liver's internal structure. When the scarring and distortion become widespread, 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 Cirrhosis of the Liver develops.

Causes of PBC

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.

Symptoms of PBC

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

The first symptoms often include

  • Itchiness

  • Fatigue

  • Dry mouth and eyes

Other problems may not occur until months or years later:

As the disorder progresses, itching may disappear and the yellow bumps may shrink.

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 Symptoms 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 Osteoporosis , 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.

Diagnosis of PBC

  • Abnormal liver function tests

  • Antibodies to mitochondria

  • Imaging tests

  • Biopsy

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, hepatitis in donated blood) and to measure the severity and progress of... read more are abnormal.

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, radionuclide scanning, 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 cholangiography) and often ultrasonography. If results of these tests are not clear, endoscopic retrograde cholangiopancreatography 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).

Prognosis of PBC

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:

Treatment of PBC

There is no known cure. Treatment includes

  • Drugs to relieve symptoms, primarily itching

  • Ursodeoxycholic acid to slow the progression of liver damage

  • Obeticholic acid is a new medication that was approved by the Food and Drug Administration (FDA) in 2016 for the treatment of primary biliary cholangitis (PBC). This medication is intended for use in people in whom ursodeoxycholic acid is not working well. It should be used with caution in people with advanced liver disease.

  • Treatment of complications

  • Eventually liver transplantation

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 is a newly FDA-approved medication which has been proven to improve liver-related blood tests in many people with PBC for whom ursodeoxycholic acid alone is not effective.

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 Osteoporosis 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 injection.

More Information about PBC

Drugs Mentioned In This Article

Generic Name Select Brand Names
Obeticholic Acid
EVISTA
ZOLOFT
REVIA
RIFADIN, RIMACTANE
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