Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic disease in which the bile ducts in your liver are slowly destroyed. Bile is a fluid made in your liver. It aids with digestion and helps your body get rid of cholesterol, toxins, and worn-out red blood cells. It’s not clear what causes primary biliary cholangitis.  Possible immunological, autoimmune, genetic, and/or environmental factors are under investigation as potential causes. Many experts consider it an autoimmune disease in which the body turns against its own cells.

Common early symptoms include:

• Fatigue
• Itchy skin
• Dry eyes and mouth

Later signs and symptoms may include:

• Pain in the upper right abdomen
• Swelling of the spleen
• Bone, muscle or joint (musculoskeletal) pain
• Swollen feet and ankles (edema)

There’s no cure for primary biliary cholangitis, but medications are available to help slow the progression of the disease and prevent complications. Options include:

• Ursodeoxycholic acid (UDCA)
• Obeticholic acid (Ocaliva)
• Fibrates (Tricor)
• Other medications
• Liver transplant.

The diagnosis of PBC requires the presence of:

1. High alkaline phosphatase (ALP, a liver blood test) along with
2. Positive anti-mitochondrial antibody (+AMA)

PBC can often go unnoticed or, more commonly, misdiagnosed, leaving patients’ symptoms to develop and cause further damage to their body. PBC affects mostly women, but more men are now being diagnosed. The disorder usually becomes apparent during middle age, initially affecting most individuals between the ages of 45 to 65 years. However, the disorder has been diagnosed in females as young as 22 years of age and in females in their early 90s. It has been estimated that PBC is one of the most common autoimmune diseases, affecting nearly 1 in 1000 women over the age of 40.