Primary biliary cholangitis

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:

• Fatigue (reported in more than 85% of patients)
• Pruritus (Intense itching, especially at night)
• Dry mouth and eyes due to the involvement of salivary and conjunctival gland

Additional symptoms may include:

• Enlargement of spleen (splenomegaly) or the liver (hepatomegaly)
• Abdominal pain on the right side, located in the liver area
• Yellowing of the skin (jaundice)
• Localized accumulation of lipid deposits under the skin, commonly near the eyelids (xanthelasma)
• Thyroid disorders, such as hypothyroidism
• Fragile bones leading to osteoporosis

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:

• Ursodeoxycholic acid (UDCA) or ursodiol: This is the primary and commonly prescribed drug. It helps to remove the bile from the bloodstream and to reduce the inflammation of the bile ducts, slowing down the progression of the disease.
• Obeticholic acid (OCA): This medication is used as an alternative treatment when the previous therapy is not effective.

Additional supportive measures to relieve the symptoms include:

• Cholestyramine, used to relieve itching.
• Supplementation for vitamin deficiencies (A, D, E y K) as well as calcium.
• Management of thyroid hormonal alterations.

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.