What is primary biliary cholangitis?

Primary biliary cholangitis (PBC) is a chronic, progressive autoimmune liver disease characterised by inflammation and gradual destruction of the small bile ducts within the liver. These ducts play a crucial role in transporting bile, a fluid necessary for digesting fats and removing waste products. When the ducts are damaged, bile accumulates in the liver, leading to inflammation, scarring (fibrosis), and potentially cirrhosis if not managed properly.

Although its exact cause remains unknown, PBC is believed to result from a combination of genetic susceptibility and environmental triggers. It occurs far more frequently in women, particularly those between the ages of 40 and 60. Family history and other autoimmune conditions—such as autoimmune thyroid disease, Sjögren’s syndrome, and rheumatoid arthritis—may increase the risk.

Many individuals with PBC are diagnosed before symptoms appear, often through routine blood tests showing elevated liver enzymes, particularly alkaline phosphatase (ALP). When symptoms do develop, the most common include persistent fatigue and pruritus (itching). Some individuals may also experience dry eyes and mouth, abdominal discomfort, or signs of advanced liver disease such as jaundice or swelling in the legs and abdomen.

Diagnosis is typically based on a combination of blood tests, including the presence of antimitochondrial antibodies (AMA), imaging to rule out other causes of bile duct obstruction, and occasionally a liver biopsy.

While there is no cure, modern treatment can significantly slow disease progression. Ursodeoxycholic acid (UDCA) is the first-line therapy and can improve liver function and long-term outcomes. For patients who do not respond adequately, there are a range of second line therapies now available to manage this condition.

This includes Bezafibrate, obeticholic acid, Elafibrinor and Seladelpar. However, a holistic approach to managing PBC is required including managing symptoms of pruritus (itch) and fatigue. Moreover, being aware of other associated conditions such as Raynaud's syndrome Sjogren syndrome and looking after bone health remain important pillars in management

With early diagnosis and appropriate treatment, many people with PBC are able to maintain good quality of life and avoid severe liver complications for many years. Regular monitoring and collaboration with a healthcare provider remain essential to long-term management.