Primary Biliary Cirrhosis

Primary biliary cirrhosis is a rare disease in which the bile ducts become progressively inflamed and destroyed. Bile builds up in the liver (cholestasis), causing fibrosis and cirrhosis of the liver.

Primary biliary cirrhosis differs from secondary biliary cirrhosis, in which the bile ducts are obstructed or damaged due to another cause such as a tumor.

Causes of primary biliary cirrhosis

The cause of primary biliary cirrhosis (also abbreviated PBC) is not fully understood but is considered an autoimmune disease, in which the immune system attacks the body’s bile duct cells. Some experts believe PBC may be triggered by a fungal, bacterial, or parasitic infection.

Women are nine times more likely than men to develop PBC.

Symptoms of primary biliary cirrhosis

Some patients experience no symptoms, while others may experience symptoms such as:

  • Fatigue
  • Itchy skin
  • Abdominal pain
  • Dry eyes
  • Dry mouth
  • Yellowing of the skin and eyes (jaundice)
  • Darkening of the skin
  • Swollen feet and ankles
  • Accumulation of abdominal fluid (ascites)
  • Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas)
  • Greasy diarrhea
  • Memory problems
  • Vitamin deficiencies

Serious complications of PBC include:

  • Cirrhosis of the liver
  • Portal hypertension
  • Varices (enlarged veins in the esophagus and stomach that are susceptible to life-threatening bleeding)
  • Osteoporosis
  • Liver cancer

Treatments for primary biliary cirrhosis

There is no cure for primary biliary cirrhosis, so treatments are aimed at slowing the progression of disease, relieving symptoms, and preventing complications.

Medications

Medications are used to reduce cholestasis, improve liver function, relieve itching and fatigue, and to treat complications such as varices and osteoporosis. The first line of treatment is Ursodeoxycholic acid (UDCA), also known as ursodiol (Actigall, Urso). Medication and surgery for portal hypertension may also be needed.

Liver Transplant

If medical therapies no longer control symptoms and the liver begins to fail, liver transplantation may be considered. Primary biliary cirrhosis can recur in the new liver after some years.