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Disease pathophysiology:
Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and obliterative fibrosis of bile ducts inside and/or outside of the liver. This pathological process impedes the flow of bile to the intestines and can ultimately lead to cirrhosis of the liver, liver failure, and other complications, including but not limited to bile duct and liver cancer.
Disease statistics:
Approximately 70% of patients with PSC are men, with a mean age of diagnosis around 40 years. Patients with PSC but without IBD are more likely to be women and to be older at diagnosis.The prevalence of PSC is estimated to be 6.3 cases per 100,000 population. The disease normally starts at age 20-30 years, although it may begin in childhood.
Disease treatment:
Over time, many PSC patients will continue to suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery.