Primary Sclerosing Cholangitis

Causes

Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The underlying cause of the inflammation is believed to be autoimmune.

Symptoms

Early symptoms often include:

• Fatigue
• Itching

Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years. Signs and symptoms that may appear as the disease progresses include:

• Pain in the upper right part of the abdomen
• Fever
• Chills
• Night sweats
• Enlarged liver
• Enlarged spleen
• Weight loss
• Yellow eyes and skin (jaundice)

Diagnosis

Tests and procedures used to diagnose primary sclerosing cholangitis include:

• Liver function blood tests.
• Magnetic resonance cholangiopancreatography
• Endoscopic retrograde cholangiopancreatography (ERCP)
• Liver Biopsy

Treatment

• Symptomatic – Supportive treatment
• Nutritional support
• Liver transplantation