Primary Biliary Cholangitis

What is Primary Biliary Cholangitis (PBC)?

Primary Biliary Cholangitis, formerly known as primary biliary cirrhosis, is a chronic autoimmune disease where your body attacks the bile ducts in your liver. Bile ducts are responsible for carrying bile, needed for food digestion, from the liver to the small intestines. Since these ducts stop working, bile starts accumulating in the liver causing inflammation and scarring. People who get PBC commonly have other autoimmune diseases such as scleroderma, Sjogren’s syndrome, hepatitis, and thyroid diseases. The main risk factors for PBC are being a woman, being 30-60 years old, having a family member with the disease, and living in North America or northern Europe. 

What are the symptoms of Primary Biliary Cholangitis?

  • Fatigue
  • Abdominal pain
  • Itchy skin
  • Muscle and joint pain
  • Dry eyes and mouth
  • Swollen knees and ankles
  • A buildup of fluid in the abdomen
  • Yellowing of skin 
  • Fatty deposits on the skin around the eyes
  • Swelling of the spleen

What are the complications of Primary Biliary Cholangitis?

  • Gallstones: since bile can’t flow through the bile ducts, it hardens and turn into stones
  • Portal hypertension: normal blood flow through your liver is blocked due to scarring, which causes blood to back up and increase in pressure. Also, since blood is not passing through your liver, medications and toxins aren’t filleted from the bloodstream. 
  • Spleen Enlargement: the spleen becomes enlarged due to an accumulation of platelets and white blood cells since the body is no longer filtering toxins by the liver.
  • Vitamin deficiencies: due to the lack of bile in the small intestines, vitamins such as vitamins A, D, E, and K can’t be absorbed.
  • Hepatic encephalopathy: brain functions declines due to the buildup of toxins in the bloodstream. This can end up in brain damage. 

How is Primary Biliary Cholangitis diagnosed?

The diagnosis of primary biliary cholangitis can be made by a history, physical exam looking for hallmark signs, and some blood tests. A blood test to measure the anti-mitochondrial antibody (AMA) can be ordered, which detects autoantibodies for this disease. Therefore, the presence of AMA is usually confirmatory by PBC. Other bloodwork such as liver enzymes, cholesterol, and triglycerides can be obtained. If the diagnosis is uncertain, a liver biopsy can be done looking for a definitive diagnosis. 

What are the differential diagnoses of Primary Biliary Cholangitis?

  • Primary sclerosing cholangitis 
  • Chronic hepatitis
  • Infiltrative liver disease. 

How is Primary Biliary Cholangitis treated?

There is not a cure for PBC. Some medications can be prescribed to slow the progression and manage the symptoms. The first-line treatment for PBC is usually ursodiol (Actigall). This medication helps remove bile from the liver, improving liver function and preventing the progression of the disease. Also, obeticholic acid (Ocaliva) can be used to reduce the amount of bile produced while increasing the bile flow in the liver. Both medications can be used in combination or alone. Benadryl is used to treat the itching and artificial tears and saliva substitutes can help improve the dry mouth and eyes. When medications don’t seem to help, a liver transplant is recommended. This is associated with very high long-term outcomes. If there is a vitamin deficiency, then vitamin supplementation should be started. 
For more information about primary biliary cholangitis follow the Liver Foundation website

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