What is primary biliary cholangitis (PBC)?

Primary biliary cholangitis (PBC) is a progressive and chronic autoimmune disease that affects the bile ducts in the liver. A long-term treatment plan that includes medicine to help slow the progression of PBC (and blood tests every 3 to 6 months to check progress) can help prevent serious health issues, including the need for a liver transplant.

What’s in a name?

  • P = Primary:
    A disease that starts spontaneously and without obvious cause
  • B = Biliary:
    Related to or affecting the bile ducts in the liver
  • C = Cholangitis:
    Inflammation of the bile ducts

Cholangitis vs cirrhosis

PBC was once called primary biliary cirrhosis, which led some people to believe PBC was alcohol related. The current name is more medically accurate and less likely to cause misunderstanding.

Important things to remember about PBC

PBC is an autoimmune disease.
  • It causes your body to attack itself
  • When the body attacks its own cells, it causes inflammation and damage
  • With PBC, your body attacks your own bile ducts in the liver
PBC is progressive and chronic.
  • PBC can get worse
  • It will not go away over time
  • With proper treatment, it’s possible to manage PBC
PBC affects your bile ducts, which play an important role in keeping your liver healthy.
  • PBC damages bile ducts and causes bile to build up in your liver
  • Bile buildup, or cholestasis, can cause harmful scarring known as fibrosis
  • Worsening fibrosis can lead to cirrhosis, which severely interferes with liver function
  • The cirrhosis people with PBC can get is unrelated to drinking alcohol

What causes PBC?

  • The exact cause of PBC is unknown; however, it is known that PBC is not caused by drinking alcohol.
  • PBC is not contagious, and it’s not your fault.
  • Scientists think PBC can be inherited. If one person in a family has PBC, other members of the family are more likely to have it as well.
  • Environmental factors, such as being exposed to cigarette smoke or certain chemicals, may play a role in causing PBC.

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