Primary Biliary Cholangitis (PBC)

Formerly known as primary biliary cirrhosis, primary biliary cholangitis inflames the small tubes in the liver (bile ducts) that carry bile from the liver to other parts of the digestive system. When bile ducts stop functioning properly, bile can build up in the liver and cause scarring (cirrhosis) that can reduce liver function. More than 90% of individuals who develop PBC are women.

Causes of Primary Biliary Cholangitis

Primary biliary cholangitis is believed to be an autoimmune disease that causes the body’s immune system to turn against its own cells. Liver inflammation from PBC starts when white blood cells collect in the liver and destroy the healthy cells that line the small bile ducts. This causes inflammation which can spread to other areas of the liver. As the affected cells die, they turn into scar tissue, which impacts the liver’s ability to work properly.

Symptoms of Primary Biliary Cholangitis

Many individuals diagnosed with this condition do not show symptoms during its early stages. If symptoms do appear, they may include fatigue and itchy skin.

Symptoms that can develop over the next few years as the disease progresses can include:

  • Dry eyes and mouth
  • Upper right abdomen pain
  • Darkening of the skin
  • Muscle or joint pain
  • Jaundice (yellowing of the skin and eyes)
  • Swollen feet and ankles
  • Swelling of the spleen
  • Diarrhea
  • Weight loss
  • Fluid buildup in the abdomen

Diagnosing Primary Biliary Cholangitis

If you’re experiencing symptoms of PBC, your medical provider will likely review your medical history and perform a physical exam to look for signs of the condition. You may also be asked to undergo blood tests to check your liver enzyme and cholesterol levels and to look for antibodies that can indicate the presence of an autoimmune disease. An imaging test, such as an ultrasound or MRI, may also be needed to rule out other conditions with similar symptoms.

Treatments for Primary Biliary Cholangitis

There is currently no cure for primary biliary cholangitis, so patients are given a variety of medications that help slow its progression and prevent complications. Additional treatments are designed to minimize symptoms and complications.

While no cure for autoimmune liver diseases such as primary biliary cholangitis exists at this time, it remains important to seek early treatment to prevent further liver damage. The team of liver disease and hepatology specialists at Tampa General Hospital stands ready to help.