Problem

Primary biliary cirrhosis

Other Names:
Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver Over time, the disease can cause cirrhosis and may make the liver stop working
Nature:

Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue can lead to scarring, fibrosis, and eventually cirrhosis.

Common symptoms are tiredness, itching and, in more advanced cases, jaundice. In early cases, there may only be changes in blood tests.

PBC is a relatively rare disease, affecting up to 1 in 3–4,000 people. It is much more common in women, with a sex ratio of at least 9:1 female to male.

The condition has been recognised since at least 1851 and was named "primary biliary cirrhosis" in 1949. Because cirrhosis is a feature only of advanced disease, a change of its name to "primary biliary cholangitis" was proposed by patient advocacy groups in 2014.

Incidence:
The cause of primary biliary cirrhosis is unknown. Some research suggests that the disease might be caused by a problem within the immune system.
Related Problems:
Sjogren's disease
Problem Type:
G: Very specific problems
Subject(s):
Date of last update
11.01.2018 – 03:11 CET