Abstract
Primary sclerosing cholangitis is a chronic progressive cholestatic liver disease affecting intrahepatic and extrahepatic biliary ducts. It leads to liver cirrhosis finally.
Strong association with inflammatory bowel disease especially ulcerative colitis is typical. Diagnosis is based on MRCP finding mainly, laboratory test and liver biopsy in some cases.
Endoscopic therapy with dilatation of dominant structure is crucial. Effect of pharmacotherapy is still discussed and ursodeoxycholic acid could be used.
Associated symptoms as pruritus and steoporosis require treatment too. During follow up patients are in the risk of bacterial cholangitis and malignant tumor development (cholangiogenic and colorectal carcinoma mainly).
In case of liver cirrhosis complications based on associated portal hypertension can occur. Liver transplatantion must be considered in such cases and in case of worsening liver functions, severe pruritus and recurrent bacterial cholangitis.