Primary Biliary Cholangitis (PBC)

Primary biliary cholangitis is a long-term autoimmune disease in which the immune system slowly damages the small bile ducts inside the liver. Bile then builds up and injures liver tissue, which over many years can lead to scarring and cirrhosis.

About 9 in 10 of those affected are women, usually diagnosed between 40 and 60. It is often found early through routine blood tests. With daily treatment most people diagnosed early have a normal or near-normal life expectancy, although itching and fatigue can remain troublesome.

Symptoms

• No symptoms in many people at diagnosis
• Tiredness that is not relieved by rest
• Itching, often worse at night and on the palms and soles
• Dry eyes and dry mouth
• Discomfort under the right ribs
• Yellowish fatty deposits around the eyelids
• Jaundice, swelling of the abdomen or legs in advanced disease

Causes and risk factors

The cause is an autoimmune attack on bile duct cells in people with a genetic susceptibility, probably set off by an environmental trigger that has not been identified. Smoking and repeated urinary infections are associated with it. It is not caused by alcohol and it is not contagious. PBC frequently occurs together with other autoimmune conditions such as Sjogren's syndrome, thyroid disease, scleroderma and coeliac disease, and sisters and daughters of patients have a somewhat higher risk.

How it is diagnosed

• Liver blood tests: A persistently raised alkaline phosphatase, often with raised GGT, is the typical pattern.
• Antimitochondrial antibodies (AMA): Present in about 95 percent. With a raised alkaline phosphatase, they are enough for the diagnosis. PBC-specific antinuclear antibodies (sp100, gp210) help in AMA-negative cases.
• Ultrasound: Excludes a blockage of the large bile ducts.
• Elastography (FibroScan): Measures liver stiffness to stage scarring without a biopsy.
• Liver biopsy: Needed only when the antibodies are negative or an overlap with autoimmune hepatitis is suspected.
• Bone density and vitamin levels: PBC increases the risk of osteoporosis and of low fat-soluble vitamins.

Treatment options

• Ursodeoxycholic acid: The first-line treatment, 13 to 15 mg per kg daily for life. It slows progression in most patients. Response is judged from blood tests after 6 to 12 months.
• Second-line medicines: Added if the response to ursodeoxycholic acid is inadequate. Options include bezafibrate or fenofibrate, the newer PPAR agonists elafibranor and seladelpar where licensed, and obeticholic acid, whose availability has been restricted in some regions.
• Treatment of itching: Cholestyramine first, then rifampicin, naltrexone or sertraline. Fibrates also help.
• Managing fatigue, dryness and bone health: Exclude other causes of tiredness, use eye drops and saliva substitutes, and take vitamin D, calcium and bone medicines as needed.
• Surveillance in cirrhosis: Ultrasound every 6 months and endoscopy for varices.
• Liver transplant: For liver failure or itching that cannot be controlled. Results are good, though PBC can return slowly in the new liver.

When it is urgent

Vomiting blood or passing black stools, new confusion or marked drowsiness, a rapidly swelling abdomen with fever, or deepening jaundice need an emergency department near you without delay.

Travelling to Türkiye for treatment

PBC is managed with tablets and periodic blood tests for life, which should be arranged with a liver specialist at home. A trip can be useful for confirming the diagnosis, staging the liver with elastography, an expert opinion when blood tests have not normalised on ursodeoxycholic acid, or transplant assessment. For transplantation in Türkiye a living related donor and ethics committee approval are required. Before starting any second-line drug abroad, check that you can obtain it at home.

Send your reports, scans and a short history and a Clinic-Y coordinator replies within 24 hours with suitable teams and written, all-inclusive proposals side by side. Reviewing your case is free.

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