[Possible treatment modalities of primary sclerosing cholangitis]

Primary sclerosing cholangitis (PSC) is a chronic progressive liver disease with the segmental inflammation, obliterative fibrosis and cholestasis of intra- and extrahepatic biliary ducts which leads to secondary biliary cirrhosis. Its aetiology is unknown. The disease is accompanied with ulcerative colitis in 70-80 percent. Its clinical progress is variable, the average duration of the disease is between the diagnosis and the final stage (liver transplantation) about 18 years. The prevalence of PSC is 1-5/100 000 inhabitants. Mainly it occurs in male patients (70 %), male-female rate is 2:1. The authors summarise the treatment modalities after reviewing the pathogenesis, clinical picture and the diagnostic procedures. The pathomechanism of the basis therapy with ursodeoxycholic acid (UDCA) is discussed in details, especially its effects on the clinical picture and the laboratory data. The authors review the results of the immunosuppressive therapy and the treatments of specific complications in PSC. They underline the importance and opportunities of the early diagnosis of cholangiocarcinoma, which is very frequent in this disease.