Primary biliary cholangitis (PBC) is a chronic inflammatory autoimmune disease that leads to a gradual and progressive destruction of the bile ducts in the liver, resulting in impaired excretion of bile, which is essential for fat digestion.
As bile acids and cholesterol cannot be properly eliminated through bile, they accumulate in the bloodstream. This leads to inflammation of the liver and, over time, can cause liver cell destruction and the development of cirrhosis. It is due to this progression that primary biliary cholangitis was previously referred to as primary biliary cirrhosis.
It is an autoimmune disease where the immune system mistakenly attacks its own tissues.
It is believed to have a genetic predisposition and may be triggered by environmental factors.
Primary biliary cholangitis (PBC) is a rare disease, with an estimated prevalence of 1 to 50 cases per million people.
It predominantly affects women, with a ratio of approximately 9 to 1 compared to men, and it usually appears between the ages of 30 and 60.
While approximately half of the patients with primary biliary cholangitis (PBC) may not experience any associated symptoms, those who do may present with the following primary symptoms:
Additional symptoms may include:
In addition to symptoms, primary biliary cholangitis (PBC) is often characterized by elevated levels of alkaline phosphatase in the blood. Furthermore, 95% of cases show positive high levels of antimitochondrial antibodies (AMA).
In certain cases, a liver biopsy may be performed as a final diagnostic procedure, although it is not always completely accurate.
The recommended treatment for primary biliary cirrhosis focus on to slowing down disease progression.
The most common used medication include:
Additional supportive measures to relieve the symptoms include:
In cases of severe liver injury, liver transplantation is considered as a potential treatment option, with a five-year survival rate of 80-85%.
Primary biliary cholangitis (PBC) has a slow and progression. The obstruction of the bile ducts predisposes to the development of cirrhosis after a span of 15 to 20 years.