FAQ

Primary biliary cholangitis (PBC) is a chronic (e.g.,long lasting), progressive liver disorder that mostly affects women and usually appears during middle age. Approximately 25% of patients with PBC are women younger than 40 years of age, and about 10% of patients are men. PBC leads to inflammation and scarring of the small bile ducts (the ‘plumbing’ system of the liver which transports bile, the substance that helps digest fat). 

When PBC is very severe, it can lead to yellow discoloration of the skin (jaundice), which occurs when bilirubin levels rise above 2 to 3mg/dL or 34 to 51 µmol/L). If PBC is untreated or there is incomplete response to medical treatment, it can lead to cirrhosis (e.g., scarring of the entire liver), which can lead to liver failure. PBC is divided into four stages from stage 1 (early disease, without significant scarring of the liver) to stage 4 (cirrhosis). Although the exact cause of PBC is unknown, it is thought that it is likely due to a combination of factors such as autoimmune (when a person’s own immune system attacks their body), genetic, and environmental factors.

90 percent of PBC patients are women, and most patients are diagnosed between the ages of 35 and 55. The prevalence of the disease (number of cases in a given population) for women above the age of 40 is estimated to be ~ 1 / 1000. PBC should thus be considered a rare disease.

The cause of this liver disease is unknown. A genetic predisposition in combination with environmental factors may be responsible for the autoimmune dysfunction triggering the development of PBC.

A rapid and precise diagnosis is essential to determine the appropriate treatment. As PBC is a rare liver disease, it is important that PBC is monitored by hepatologists (experts in diseases of the liver)

Your doctor will examine your case and prescribe you the treatment(s) that are needed, also according to your response to the treatment(s) as measured by blood tests. In addition, he/she will assess possible problems which may occur during the course of the disease, including other conditions related to PBC.

The standard first line treatment for PBC is ursodeoxycholic acid (UDCA), which is usually very well tolerated. The preferred dosage is between 13 and 15 mg per kilogram of body weight per day. Your hepatologist will calculate the correct dosage for you, which may change over time in case of significant weight changes. As adequate dosages of UDCA are associated with prolonged life expectancy and a lower rate of liver transplantation in PBC, it can be considered essential to protect your liver. Indeed, in most patients, the progressive damage to the liver can be effectively stopped/slowed down by UDCA.