Primary biliary cholangitis (PBC)

Primary biliary cholangitis (PBC; formerly known as primary biliary cirrhosis) is a rare, chronic autoimmune liver disease. It affects women significantly more often than men.

In PBC, the immune system primarily attacks the small bile ducts in the liver. This can cause bile to build up and the liver tissue to become inflamed. Without treatment, the disease may lead to progressive scarring of the liver (fibrosis) and eventually to cirrhosis. If PBC is diagnosed early and treated effectively, its progression can be significantly slowed in many people.

Typical findings include elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) levels. Antimitochondrial antibodies (AMA) are also frequently detected in the blood. Elevated bilirubin may indicate a more advanced stage of the disease and can cause yellowing of the skin and eyes.

Causes

The exact cause of PBC is not fully understood. A combination of genetic predisposition, changes in the immune system and environmental factors is thought to play a role. PBC is not contagious and is not caused by alcohol consumption.

Symptoms

PBC may remain without noticeable symptoms for a long time. Common symptoms include:

• tiredness and exhaustion (fatigue)
• itching
• dry eyes, dry mouth and dry mucous membranes
• joint, muscle or bone pain
• sleep disturbances and restless legs
• difficulty concentrating

Symptoms vary considerably and do not always correspond to the stage of the disease.

Regular physical activity adapted to the individual’s abilities, good sleep habits and sufficient rest can help with fatigue. Other possible causes, such as anaemia, thyroid disorders, sleep disorders or vitamin deficiencies, should also be investigated.

Several medications are available to treat itching. The appropriate treatment must be determined individually and monitored by a physician.

PBC may also be associated with osteoporosis, elevated blood lipid levels and other autoimmune diseases. Additional examinations, such as bone density measurements and checks of thyroid function or vitamin levels, may therefore be useful.

Diagnosis

If PBC is suspected, liver values and antimitochondrial antibodies are tested. The diagnosis can often be made based on the typical laboratory findings.

A liver biopsy is not always necessary. It may be useful if the findings are inconclusive or if an additional liver disease is suspected. Ultrasound and elastography can help assess possible scarring of the liver.

Treatment

PBC cannot currently be cured. Treatment aims to slow the progression of the disease, relieve symptoms and prevent complications. Alkaline phosphatase and bilirubin are particularly important for assessing the response to treatment.

The standard treatment is ursodeoxycholic acid (UDCA). It is taken daily, at a dose adjusted to body weight, and usually on a permanent basis. After approximately six to twelve months, the response to treatment is assessed.

If UDCA is not sufficiently effective or is not tolerated, additional or alternative treatments may be considered.

Seladelpar (Lyvdelzi®) was temporarily authorised by Swissmedic on 9 December 2025 for adults with PBC. It can be used with UDCA if UDCA alone is not sufficiently effective, or as monotherapy if UDCA is not tolerated. The authorisation is based on a reduction in alkaline phosphatase. Its long-term benefits require further investigation. In some people, seladelpar may also relieve itching. Information from Swissmedic(opens in new tab)

Elafibranor (Iqirvo®) is authorised in the European Union and the United States, but is not currently authorised in Switzerland. In individual cases, the treating specialist may apply for reimbursement of the imported medicine under Article 71c of the Swiss Health Insurance Ordinance. Prior approval from the health insurer is required before treatment begins.

Fibrates, such as bezafibrate, may improve liver values and, in some people, itching when the response to UDCA is insufficient. As they are not authorised for PBC in Switzerland, they are used off-label. Regular monitoring is required because of possible effects on liver values, muscles and kidney function.

Obeticholic acid (Ocaliva®) is no longer authorised in Switzerland. In August 2025, Swissmedic announced the withdrawal of its authorisation after clinical benefit was not demonstrated in a confirmatory study. No new treatments should be started outside clinical trials. People already treated with obeticholic acid should discuss possible alternatives with their specialist. Information from Swissmedic(opens in new tab)

Immunosuppressive medicines are generally not used for PBC alone. They may be required if autoimmune hepatitis is also present. This is known as PBC-AIH overlap syndrome.

Symptoms and associated conditions such as itching, fatigue, dry mucous membranes or osteoporosis should be treated specifically. Regular specialist follow-up is important. Alcohol should be avoided as far as possible, and smoking is not recommended.

Liver transplantation

In advanced disease, a liver transplant may become necessary. PBC-specific antibodies may remain detectable in the blood after transplantation. In some transplant recipients, PBC-related changes may develop again in the new liver. Regular follow-up therefore remains necessary.

Treatment must be determined individually by a liver specialist. Authorisation of a medicine does not automatically mean that its costs will be covered by the health insurance provider.