Primary Biliary Cholangitis


The exact mechanism of the liver damage is unknown, although evidence indicates that it can be of autoimmune origin. Primary biliary cholangitis is a disease of unknown etiology, but various factors have been implicated as the causes of this illness, such as the following:

  • Genetic factors: First-degree relatives have a 570- to 1000-fold increased chance of developing primary biliary cholangitis.
  • Infection with organisms of the family Enterobacteriaceae: Cross-reactivity between antigens on the bacterial wall and the mitochondria has been postulated.

Symptoms and Signs

About half of patients present without symptoms. Symptoms or signs may develop during any stage of the disease and may include fatigue or reflect cholestasis (and the resulting fat malabsorption, which may lead to vitamin deficiencies and osteoporosis), hepatocellular dysfunction, or cirrhosis. Pruritus, fatigue, and dry mouth and eyes are the initial symptoms in  of patients and can precede other symptoms by months or years. Other initial manifestations include right upper quadrant discomfort (10%); an enlarged, firm, nontender liver (25%); splenomegaly (15%); hyperpigmentation (25%); xanthelasmas (10%); and jaundice (10%). Eventually, all the features and complications of cirrhosis occur.


  • Liver tests
  • Antimitochondrial antibodies (AMAs)
  • Ultrasonography and often magnetic resonance cholangiopancreatography (MRCP)
  • Liver biopsy


There’s no cure for primary biliary cholangitis, but medications are available to help slow the progression of the disease and prevent complications. Options include:

  • Ursodeoxycholic acid. 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.
  • Obeticholic acid. This is the newest medication approved by the Food and Drug Administration for primary biliary cholangitis.
  • Fibrates. Researchers aren’t exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. But, when taken with UDCA, it has reduced liver inflammation and itching in some people. More studies are needed to determine long-term benefits.
  • Other medications. Many other drugs have been used or studied for treating primary biliary cholangitis with mixed results. They include methotrexate and colchicine.
  • Liver transplant. When medications no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. Liver transplantation is associated with
    very good long-term outcomes for people with primary biliary cholangitis. However, sometimes the disease comes back several years later in the transplanted liver.