Primary Biliary Cirrhosis

What is it?
It 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.

When the bile ducts are damaged, bile can back up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).
Primary biliary cholangitis is considered an autoimmune disease, which means your body’s immune system is mistakenly attacking healthy cells and tissue.

What are the symptoms?
Common early symptoms include: fatigue, itchy skin, dry mouth
Later signs and symptoms may include: pain in the upper right abdomen, buildup of fluid in the abdomen due to liver failure (ascites), fatty deposits (xanthomas) on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees, yellowing of the skin and eyes (jaundice), diarrhea, which may include greasy stools (steatorrhea), weight loss

How is it diagnosed?
Your provider may order the following tests to aid in the diagnosis of PBC

  • Liver function tests
  • Antibody tests for signs of autoimmune disease
  • The following imaging studies may also be ordered:
  • Ultrasound
  • Magnetic Resonance Cholangiopancreatography (MRCP)
  • Endoscopic Retrograde Cholangiopancreatography (ERCP)

How is it treated?
There’s no cure for primary biliary cholangitis, but medications are available to help slow the progression of the disease and prevent complications.
Ursodeoxycholic acid (UDCA), also known as ursodiol (Actigall, Urso), is a medication that is commonly used first. It helps move bile through your liver. UDCA doesn’t cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. It’s less likely to help with itching and fatigue. Side effects may include weight gain, hair loss and diarrhea.
When medications no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life.

Are there any complications?

  • an enlarged spleen
  • gallstones
  • high cholesterol levels
  • weakened bones (osteoporosis)
  • vitamin deficiencies
  • cirrhosis
  • liver failure