{"id":1738,"date":"2024-08-22T15:18:30","date_gmt":"2024-08-22T21:18:30","guid":{"rendered":"http:\/\/liver.ca\/resource\/primary-biliary-cholangitis\/"},"modified":"2026-04-07T09:08:19","modified_gmt":"2026-04-07T13:08:19","slug":"cholangite-biliaire-primitive","status":"publish","type":"resource","link":"https:\/\/liver.ca\/fr\/resource\/primary-biliary-cholangitis\/","title":{"rendered":"Cholangite biliaire primitive"},"excerpt":{"rendered":"","protected":false},"author":1,"featured_media":0,"template":"","meta":{"_acf_changed":false},"resource-topic":[47],"resource-type":[73],"class_list":["post-1738","resource","type-resource","status-publish","hentry","resource-topic-primary-biliary-cholangitis","resource-type-web"],"acf":{"type":73,"topic":[47],"blocks":[{"acf_fc_layout":"text","text":"Formerly known as Primary biliary cirrhosis, PBC is a disease that affects predominantly women aged 40-60. Thanks to improvements in our ability to treat and manage PBC, less than 50 percent of patients progress to cirrhosis. Because of this fact and the issue of \u2018cirrhosis\u2019 often being connected solely to alcohol, PBC patients around the world pushed for a name change. Hepatologists agreed with the need to change the name to more accurately reflect the nature of the disease.\r\n\r\nPrimary biliary cirrhosis is now known as primary biliary cholangitis. It permits the continued use of the short form PBC while providing a more accurate description of the disease. This change has been endorsed by Liver Canada, the Canadian Association for the Study of the Liver (CASL) as well as PBC patient groups and professional associations of liver specialists worldwide.\r\n\r\nWhat is Primary Biliary Cholangitis?<\/strong>\r\n\r\nPrimary biliary cholangitis, formerly known as primary biliary cirrhosis, is a chronic liver disease. When a person has PBC, the immune system attacks the liver causing slow, progressive damage to the bile ducts (small tubes through which bile flows out of the liver).\r\n\r\nBile<\/strong>\u00a0is a yellow-green fluid produced by your liver to aid digestion. When the bile ducts are damaged, bile and other substances cannot be eliminated and accumulate in the liver. The retained substances are harmful to the liver and worsen the inflammation which leads to further damage. This can over time result in scarring of the liver. When there is a lot of scarring in the liver this is called cirrhosis.\r\n\r\nThe disease is called primary biliary cholangitis (PBC) because the immune system attacks the bile ducts inside the liver, which damages them. When immune cells surround and attack parts of the body, this is called inflammation. Inflammation of the bile ducts is called cholangitis. The medical term \u201cprimary\u201d in this case means that there is no known reason for this damage to happen. Because the disease blocks or prevents the flow of bile, PBC is known as a \u201ccholestatic\u201d liver disease.\r\n
\"A<\/a>
Used with permission from Intercept Pharma. All rights reserved<\/figcaption><\/figure>\r\n

Who is at Risk?<\/strong><\/h2>\r\nPBC generally affects women between the ages of 40 and 60, but it has been diagnosed outside of this age range, as well as in men. Nine out of 10 people who get PBC are women. Although there is no evidence that PBC is inherited from mother to child, it is more common in the daughters of women with PBC.\r\n

Cause<\/strong><\/h2>\r\nThe cause(s) of PBC remains unknown, but current evidence suggests that PBC occurs in genetically predisposed people after exposure to an undetermined environmental factor. It is important to note that PBC is not caused by alcohol consumption.\r\n\r\nPBC is believed to be an autoimmune disease. In the case of PBC, your immune system which is designed to protect you against infections and cancer mistakes the bile duct cells as being not a normal part of the body and attacks them.\r\n\r\nSomething may trigger your body into making this mistake. Possible causes of this \u201ctrigger\u201d could be an infection or some form of toxin exposure from the environment.\r\n\r\nPBC usually develops and progresses slowly. Medication can slow its progression, especially if treatment begins early.\r\n

Could I Have It?<\/strong><\/h2>\r\nMany people with PBC have no symptoms. However, when they do, symptoms may vary and may be present in any combination and include:\r\n