What is Primary Biliary Cirrhosis?
Primary biliary cholangitis or Primary Biliary Cirrhosis (PBC) is a chronic autoimmune hepatitis that gradually destroys small bile ducts in the liver resulting in the accumulation of bile in the liver and possible liver cell damage in the liver.
What are the common symptoms of PBC?
Early on, you may feel well, but as PBC progresses you can experience intense itching, fatigue, dry eyes or mouth, dark urine, light-colored stools, and discomfort or pain in the upper right abdomen.
What causes Primary Biliary Cirrhosis?
It is thought to be caused by an autoimmune response to bile ducts, but the exact mechanism of its pathogenesis remains unclear, and the risk factors that lead to PBC are related to family history of liver disease and some infections or chemical exposures, and the preponderance of female individuals between 30-60.
How is PBC diagnosed?
Diagnosis involves blood tests to check liver enzymes and antimitochondrial antibodies (AMA), imaging studies such as ultrasound or elastography, and in some cases a liver biopsy to assess the extent of bile duct injury.
Is PBC the same as primary biliary cholangitis?
Yes. Primary biliary cholangitis is a new name of the disease which was once known as primary biliary cirrhosis. Both of the names are the same underlying autoimmune disease of the bile ducts.
Can PBC cause joint pain?
Yes. Because PBC is an autoimmune disorder, it can be associated with other autoimmune conditions and may cause symptoms such as joint pain or stiffness in some patients.
How is itching managed in PBC?
Drugs such as cholestyramine, antihistamines, or rifampicin can be used to relieve itching (pruritus). Therapy will be different according to the severity of symptoms and side effects depending on your doctor.
What dietary and lifestyle changes help manage PBC?
A balanced, low-salt diet rich in fruits, vegetables, and lean proteins is recommended. Avoiding alcohol, maintaining a healthy weight, exercising regularly, and taking fat-soluble vitamin supplements (A, D, E, K) can support liver health.
What are the main treatments for PBC?
The treatment of the first-line is ursodeoxycholic acid (UDCA) to facilitate bile flow. In case of a poor response, obeticholic acid can be added. It is treated through the use of endurance following blood tests and blood imaging to go further.
When might a liver transplant be necessary?
When PBC arrives in spite of the progress in the optimum medical treatment and causes liver failure or life threatening complications, then, liver transplant must also be considered to help in restoring the liver functions and guaranteeing survival.
Medically Reviewed by
Dr. Bharat Pothuri, MD, FACG
| Updated 03-17-2026