Primary sclerosing cholangitis

Primary Sclerosing Cholangitis

Overview

Primary sclerosing cholangitis is an autoimmune condition leading to inflammation and damage of the bile ducts. When the bile ducts get damaged, bile made in the liver cannot reach the small intestine to digest fats. The backed-up bile can cause inflammation and scarring in the liver. It is a chronic, progressive disease and can co-exist with certain autoimmune conditions of the intestine. It is diagnosed commonly in older children and young adults.

Signs and symptoms

Itching, episodes of jaundice with fever, weight loss, and fatigue are common early symptoms. As the condition progresses, all features of liver failure can develop. This condition is also associated with an increased risk of tumors in the liver and the bile ducts.

Causes and risk factors

Primary sclerosing cholangitis is considered an auto-immune condition. An immune response to an infection or a toxin occurs in genetically predisposed patients, triggering an auto-immune attack on the bile ducts. Patients with inflammatory bowel disease such as ulcerative colitis have a higher chance of developing this condition.

Complications

Complications include liver failure, repeated liver or bile duct infections, portal hypertension and an increased risk of colon and bile duct cancer.

Diagnosis

Primary sclerosing cholangitis is diagnosed based on patient’s medical history and examination, liver function tests, auto-antibody levels and scans to examine the liver and the bile ducts. A liver biopsy may also be required.

Treatment and Surgical Interventions

There are currently no accepted drug therapies used to treat primary sclerosing cholangitis. Most medical interventions focus on minimizing complications and relieving symptoms. If the bile ducts are blocked, the doctor may opt to endoscopically re-open them and restore function. Extreme cases may require a liver transplant.

Prevention

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

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