Primary biliary cholangitis (PBC)

Primary biliary cholangitis (PBC) is a long-term autoimmune condition in which the small bile ducts inside the liver are slowly damaged. Bile then builds up in the liver, and over many years this can cause scarring and cirrhosis. It used to be called primary biliary cirrhosis, but most people diagnosed today do not have cirrhosis.

It mainly affects women, usually from their 40s onwards. It often occurs together with thyroid disease or dry eyes and mouth (Sjögren's syndrome).

Symptoms

• Often none: many people are found to have it after a routine blood test shows a raised alkaline phosphatase (ALP)
• Tiredness
• Itching, often worse at night
• Dry eyes and dry mouth
• In later stages, yellowing of the eyes or skin (jaundice)

Diagnosis

• Blood tests: liver tests, especially ALP, and the anti-mitochondrial antibody (AMA), which is positive in most people with PBC
• Ultrasound to rule out a blockage of the larger bile ducts, such as a gallstone
• FibroScan to measure liver stiffness and follow it over time
• Liver biopsy is needed only when the diagnosis is uncertain, for example when the antibody is negative or autoimmune hepatitis may also be present

Treatment

• Ursodeoxycholic acid (UDCA): a daily tablet taken long term. It improves bile flow and slows the disease. Blood tests after about a year show how well it is working.
• Second-line medicines are added if the blood tests do not improve enough on UDCA alone.
• Treating symptoms: medicines for itching, and eye drops or saliva substitutes for dryness
• Bone health: PBC can thin the bones, so a bone density scan, calcium and vitamin D are often advised
• If cirrhosis develops: six-monthly liver cancer checks, and assessment for a liver transplant in advanced disease

With early diagnosis and treatment, many people with PBC have a normal or near-normal life expectancy. Regular follow-up with blood tests is important.

General information only. Reviewed by Dr Chia Chung King, 2026-11-07. Book a consultation.