cirrhosis : Symptoms, Causes, Treatment & Prevention

What is Primary Biliary Cirrhosis?

Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic disease in which the bile ducts in your liver are slowly destroyed. Bile is a fluid made in your liver. It aids with digestion and helps your body get rid of cholesterol, toxins and worn-out red blood cells.

What causes Primary Biliary Cirrhosis?

The cause of PBC is unknown. It is most likely an autoimmune disease, where the body's immune system attacks its own cells. In this disease, the bile ducts are under attack and are destroyed.

What are the symptoms of Primary Biliary Cirrhosis?

Usually, primary biliary cirrhosis 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:

• The skin may become dark or nerves may be damaged (called neuropathy).
• People may feel discomfort in the upper abdomen.
• Fat sometimes accumulates in small yellow bumps in the skin (xanthoma) or eyelids (xanthelasma).

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

• Jaundice (yellowish discoloration of the skin and whites of the eyes) may develop.
• Fluid can accumulate within the abdomen (called ascites) or in other parts of the body, such as the ankles and feet (called edema).

How is Primary Biliary Cirrhosis diagnosed?

• 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 function tests) are abnormal. During the physical examination, the doctor may feel an enlarged, firm liver or an enlarged spleen. If primary biliary cirrhosis is suspected, doctors do liver function tests, imaging tests, and blood tests to check for antibodies to mitochondria.

How is Primary Biliary Cirrhosis treated?

There is no known cure. Treatment includes

• Drugs to relieve symptoms, primarily itching
• Ursodeoxycholic acid to slow the progression of liver damage
• Obetacholic acid is a new medication which was approved by the FDA in 2016 for the treatment of PBC. This medication is intended for use in patients in whom ursodeoxycholic acid is not working well.
• 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.