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Disease pathophysiology:
Primary Sclerosing Cholangitis is a rare liver and bile duct disease which may have an autoimmune component. It's not curable, but some of the symptoms are treatable - and there's hope. PSC is often associated with ulcerative colitis, Crohn’s disease, and related gastrointestinal and autoimmune diseases. Some PSC patients eventually require a liver transplant.
Disease statistics:
The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9 to 1.31 in 100,000 and point prevalence of 8.5 to 13.6 in 100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus.
Disease treatment:
Treatment of itching and osteoporosis in PSC is similar to that used for treating Primary Biliary Cirrhosis. Alendronate is a medication often used to decrease further bone loss that may help in PSC. Many medications have been studied but none have proven to alter the course of PSC. This is primarily due to uncertainty regarding its cause.