Primary biliary cholangitis (prev. Primary biliary cirrhosis)

Primary Biliary Cholangitis

Overview

Primary biliary cholangitis, formerly called primary biliary cirrhosis, is a disease that causes slow but persistent damage to the bile ducts within the liver. When the bile ducts get damaged, bile made in the liver cannot reach the bloodstream to digest fats. The backed-up bile can cause inflammation and scarring in the liver. Primary biliary cholangitis is considered an auto-immune condition. It is caused by the body’s immune system launching an attack on the bile ducts.

Signs and symptoms

Many patients diagnosed with primary biliary cholangitis experience no symptoms. However, some of the common symptoms that patients experience in the early stages are dry eyes and mouth, severe and persistent itching and extreme fatigue. In the late stages, the condition can cause upper abdominal pain, spleen enlargement, swelling of ankles and feet, jaundice, diarrhoea and weak bones.

Causes and risk factors

Primary biliary cholangitis is considered an auto-immune condition where the lymphocytes attack one’s own bile duct lining causing progressive damage. It is more common in women between the age of 30 to 60, while family history also plays a role in increasing this risk. Environmental factors that could trigger primary biliary cholangitis include smoking, infections and exposure to toxic chemicals.

Complications

In late stages, primary biliary cholangitis can cause portal hypertension, liver cirrhosis, enlargement of the spleen, gallstones in the bile duct, oesophageal varices, and hepatic encephalopathy. The condition also increases your risk of osteoporosis, liver cancer and vitamin deficiencies.

Diagnosis

Primary biliary cholangitis is diagnosed based on a patient’s medical history, a physical exam and a blood tests panel for liver function and antibodies. The doctor may also require scans to ascertain the degree of liver damage and rule out other conditions with similar symptoms.

Treatment and Surgical Interventions

There is no cure for primary biliary cholangitis. However, further progress and complications can be slowed down using drug therapies. If medications are ineffective and the liver is compromised, the doctor may recommend a liver transplant surgery, which has shown good long-term outcomes for patients with this condition. In addition to these, the doctor may recommend diet and lifestyle changes to help cope with the disease.

Prevention

Primary biliary cholangitis cannot be prevented as the cause is uncertain.

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