Primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis (PSC) represents a cholestatic long-waging liver disease, well characterised by inflammation of the bile ducts, which leads to scar formation and narrowing of the ducts over time. The occurrence differs according to geographic conditions. The primary cause of PSC remains unclear. Contemporarily it is supposed to be an autoimmune disease appearing in a field of predisposing gene mutation. The establishment of PSC is based on unique radiological and histological signs in addition to clinical and laboratory signs. The therapy of PSC can be classified from many points of view: specific therapy, the therapy of the complications, pharmacological therapy, endoscope, and surgery. Specific therapy is focused on preventing the progression of the disease. The patients with PSC make up 10 % of all transplantation candidates [7]. The results of this type of treatment are really successful, 85% of the patients outlast more than one year. Cholangitis and cholangiocellular carcinoma are included to be the most feared complication. Cholangiocellular carcinoma develops in 7-13 % of the patients with PSC [8], most frequently after long-lasting cirrhosis and in combination with colitis ulcerosa. The prognosis of this disease is very poor. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, and the majority of whom will develop progressive disease, have a survival rate greater than 70 % at 16 years after diagnosis.