It is a disease characterized by progressive inflammation and fibrosis, resulting in biliary cirrhosis in half of symptomatic patients and cholangiocarcinoma in approximately 30%.
Three quarters of the patients are male and the mean age is 40 years. It is closely related to inflammatory bowel diseases, especially ulcerative colitis. Ulcerative colitis was found in 87% and Crohn’s disease in 13% of primary sclerosing cholangitis patients with inflammatory bowel disease (IBD). 75% of PSC patients are associated with IBD.
It is not a classic autoimmune disease; but it is an immune system medical treatment disease. Scientific research shows that the average survival from the time of diagnosis is 9-12 years. Liver transplantation performed in the terminal period adds approximately another 5 years to survival.
Cholangiography with ERCP is the “gold standard” in the diagnosis of PSC. Magnetic resonance cholangiopancreaticography (MRCP) replaces ERCP in the diagnosis of PSC. MRCP gives 90% specific and sensitive results.