PBC – Primary Biliary Cholangitis

What Is PBC (Primary Biliary Cholangitis)?

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a rare chronic liver disease. PBC is a progressive autoimmune disease that slowly destroys the bile ducts (called the intrahepatic bile ducts) in your liver. Bile helps with digestion and rids your body of bad cholesterol, toxins, and worn-out red blood cells. This disease can also lead to serious issues within the liver. If the bile ducts are damaged or destroyed then bile can build up in your liver and scar the liver. If the liver becomes too damaged it could lead to liver cirrhosis.

What Causes PBC?

PBC is an autoimmune disease. What this means is that the body reacts poorly to bacteria or infection and begins to attack healthy cells and tissues.

The liver inflammation is caused when white blood cells called T cells (T lymphocytes) start to collect and attack healthy cells in the liver.

Inflammation in the small ducts spread to other parts of the liver, killing those cells and causing scarring. As the liver scars more and more it begins to function improperly, leading to cirrhosis. It is unclear what causes these white blood cells to attack healthy cells but it appears to be genetic.

Risk Factors

Some of the risk factors associated with having PBC include:

  • Being a woman
  • Age 30 to 60 years
  • Genetics – you are more likely to get PBC if a family member has had it
  • Geography- PBC is most common in northern Europe and North America
  • Smoking and other toxic chemicals
  • Certain infections

What are the Symptoms of PBC?

Often, symptoms of PBC do not manifest until five to twenty years following diagnosis. The diagnosis of PBC usually occurs while testing for other conditions.

Early symptoms of PBC include:

  • Fatigue
  • Dry mouth and eyes
  • Itchy skin

Other common symptoms of PBC include:

  • Swollen feet and ankles
  • Joint pain
  • Muscle soreness
  • Jaundice (yellowing of the skin or eyes)
  • Dark skin not related to sun exposure
  • High cholesterol
  • Dark and greasy stools
  • Brittle bones
  • Pain in the upper right abdomen

It is important to diagnose PBC as early on as possible. If you believe you may be at risk of having PBC due to family history, or any combination of the above symptoms, contact your nearest gastroenterologist today.

What are the Treatments for PBC?

There is currently no cure for PBC. Treatment focuses on slowing the progression of the disease and treating the complications. Some of the common treatment options include:

  • Exercise
  • Vitamin supplements
  • Ursodeoxycholic acid (helps move bile through your liver)
  • Fibrates (reduces liver inflammation and itching)
  • Obeticholic acid (improves liver function)
  • Liver transplant

Is PBC a Fatal Condition?

The average life expectancy of a person with PBC after they start showing symptoms, is 10 years, unless they receive a liver transplant.