Primary Biliary Cholangitis

 PBC undergoes a name change

Primary biliary cirrhosis, otherwise known as PBC, is a disease that affects predominantly women aged 40-60. Thanks to improvements in our ability to treat and manage PBC, less than 50 per cent of patients progress to cirrhosis. Because of this fact and the issue of ‘cirrhosis’ often being connected solely to alcohol, PBC patients around the world pushed for a name change. Hepatologists agreed with the need to change the name to more accurately reflect the nature of the disease.

Effective immediately, primary biliary cirrhosis will be known as primary biliary cholangitis. It permits the continued use of the short form PBC while providing a more accurate description of the disease.

This change has been endorsed by the C anadian Liver Foundation, the Canadian Association for the Study of the Liver (CASL) as well as PBC patient groups and professional associations of liver specialists world-wide.

Click here to read more about the background behind this change. 

What is primary biliary cholangitis? 
Primary biliary cholangitis (PBC) (formerly known as primary biliary cirrhosis) is a chronic liver disease. It generally strikes women between the ages of 40 and 60, but it has been diagnosed outside of this age range as well as in men. While PBC is not considered to be hereditary, the disease can be seen occasionally in siblings.

What causes PBC?
At the present time, the exact cause is unknown; however, it is known that PBC is not caused by alcohol. It is believed that PBC results from an abnormal reaction of the body's immune system possibly initiated by an infection. Designed to protect the body from infection, the immune system of PBC patients attacks the liver causing slow, progressive damage to the bile ducts (small tubes through which bile flows out of the liver). When the bile ducts are damaged, bile and other substances can not be eliminated and accumulate in the liver. The retained toxic substances and inflammation cause further damage. This eventually results in scarring of the liver (cirrhosis).
What are the symptoms of PBC?

The initial symptoms vary among PBC patients. They may be present in any combination and include:

  • mild to disabling chronic fatigue
  • intense and unrelenting itching of the skin
  • gradual darkening of the skin
  • small, white bumps under the skin, usually around the eyes.

Other symptoms, not directly related to PBC, are also often reported by patients:

  • dry membranes (nose, eyes, mouth, vagina)
  • thyroid problems
  • arthritis
  • hands that change colour in the cold (Raynaud’s disease).

As the liver damage progresses, other symptoms may appear, which affect the rest of the body. These symptoms include:

  • enlarged abdomen from fluid accumulation (ascites)
  • easy bruising or bleeding
  • jaundice (yellowing of the skin and eyes)
  • internal bleeding (gastrointestinal bleeding)
  • bone pain or spontaneous fracture can also occur due to osteoporosis.
How is PBC diagnosed?

Diagnosis is made with special blood tests including liver bichemistry. One such test detects the presence of antimitochondrial antibodies (AMA) in blood. Also, a liver biopsy  may be performed. This is a procedure in which a small needle is inserted into the liver to take a sample of the tissue for analysis. 

What is the prognosis?

The prognosis of PBC varies greatly from one patient to another. Many lead active lives with few symptoms for 10 to 20 years. In some patients, however, the condition progresses more rapidly and liver deterioration may occur in just a few years.

How is PBC treated?

There is currently no known specific treatment for PBC. Vitamins A, D and K can be prescribed, usually when jaundice is present; cholestyramine and antihistamines can successfully relieve itching.  Ursodeoxycholic acid, a bile salt, has been shown to improve liver function and delay the development of fibrosis (scar tissue) and the need for liver transplantation. When ursodeoxycholic acid is given to individuals with early disease then survival becomes the same as that of individuals of the same age without PBC.

Complications of liver deterioration can often be controlled. As an example, a salt-restricted diet and medication (usually water pills) can be prescribed following accumulation of fluid.

Liver transplantation is now a common treatment option for people with advanced PBC.

What precautions should be taken?

Alcohol may increase damage to the liver and should not be consumed regularly by anyone with any liver disease. Patients should eat a healthy and well balanced diet.

Are lifestyle changes required for PBC sufferers?

There is a healthy way to live with a chronic disease and patients with PBC should be encouraged to maintain an active lifestyle under the supervision of their doctor.

It is important for PBC patients to realize that support is available that may help them to improve their quality of life.

For more information about PBC or any other liver disease, please contact us.
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