Primary biliary cirrhosis

Primary biliary cirrhosis is a liver disease caused by autoimmune responses. In primary biliary cirrhosis, the body's own antibodies destroy the small ducts inside the liver, which consistently cause toxins and bile to accumulate in the ducts as they are damaged - this can lead to scarring in the liver which in turn can lead to liver dysfunction and potential liver failure.

Symptoms of primary biliary cirrhosis

The disease causes symptoms associated with liver problems, such as fatigue, itching and jaundice - it can also lead to other symptoms caused by increased cholesterol levels (due to the liver not being able to process these if it is damaged).

If the condition is not treated, the symptoms will gradually worsen - and can lead to complete liver failure. In later stages, fluid accumulation in the stomach, enlarged spleen, esophageal lesions and other serious symptoms of liver failure may be seen.

Clinical signs

As mentioned above under 'symptoms'.

Diagnosis and cause

The diagnosis is made through a number of clinical examinations (including blood tests), lab tests, urine analysis and a thorough history. Certain antibodies are especially looked for in the blood sample. The condition requires a liver biopsy in order to be completely sure of the stage of the disease.

Who is affected by the disease?

Primary biliary cirrhosis affects 1 per 4000 - and is far more common than you might think. Like most other liver diseases, women are affected more often than men (9: 1). The condition is most often diagnosed in the 50s.

Treatment

Drug treatment can slow the symptoms, but there is no cure for this disease. Liver transplantation may be necessary if the condition has led to liver failure.

The most common form of treatment for autoimmune conditions is included immunosuppression - that is, drugs and measures that limit and cushion the body's own defense system. Gene therapy that limits inflammatory processes in immune cells has shown great progress in recent times, often in combination with increased activation of anti-inflammatory genes and processes.

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Also read: - Complete overview of autoimmune diseases

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