Primary sclerosing cholangitis

Most individuals diagnosed with this condition are asymptomatic. Suspicion of diagnosis should arise in patients who have abnormal liver function tests with a cholestatic pattern. The majority also have concurrent inflammatory bowel disease, typically presenting with mild intestinal symptoms and, most often, mild total colitis.

Clinical Presentation:

• Asymptomatic patients with abnormal liver function tests
• Itching, fatigue, jaundice
• Episodes of cholangitis with fever
• Complications of chronic liver disease, such as esophageal varices and ascites

The diagnosis is made through cholangiography. MRCP (Magnetic Resonance Cholangiopancreatography) reveals typical changes, showing multiple focal strictures and caliber variations in the intrahepatic and/or extrahepatic bile ducts. Ultrasound/CT scans, typically accompanied by MRCP of the abdomen and repeated liver function test results, are also used in the diagnostic process.

The purpose of treatment is to:

• Prevent or treat complications such as cholangiocarcinoma, colon cancer, bleeding from stomal varices, dominant bile duct stricture.
• Reduce symptom burdenInhibit disease progression
• Optimize timing for liver transplantation