Primary Sclerosing Cholangitis
Primary sclerosing cholangitis is slowly progressive liver disease that damages the bile ducts. It is characterised by inflammation, scarring and narrowing of the bile ducts. As the disease progresses, bile builds up in the liver damaging the cells. Scar tissue can spread throughout the liver, causing cirrhosis and liver failure. The cause of primary sclerosing cholangitis is unknown, but is associated with inflammatory diseases such as inflammatory bowel disease, ulcerative colitis and Crohn disease.
Symptoms of primary sclerosing cholangitis include fatigue, itching and jaundice. Pain and fever can develop due to infection in the bile ducts. Complications include deficiencies of fat-soluble vitamins, cirrhosis (liver scarring), liver failure and bile duct cancer.
Primary sclerosing cholangitis can be diagnosed with the help of blood tests (to detect levels of liver enzymes), liver biopsy, and different imaging tests, such as ultrasound, endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiogram (PTC) and magnetic resonance cholangiopancreatography (MRCP), to confirm the diagnosis.
Treatment is aimed at relieving symptoms and includes administration of medication for itching, antibiotics for infection, and vitamin supplements. Liver transplantation may be performed if liver failure occurs.