The cause of PSC is unknown. Studies suggest impairment of the immune system or chronic infections as likely causes. Although it appears possible, it is not known if heredity plays a role, that is, if a person can inherit PSC if other members in the family are affected. Bile ducts function as the liver’s plumbing system and allow bile to flow out of the liver.
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.
Therapy is directed towards treating symptoms, preventing and managing complications of PSC. Patients with PSC should avoid anything that may cause or worsen liver damage such as alcohol. Vaccinations for hepatitis A and B should be administered. Vitamin supplements should be prescribed for patients with advanced disease at risk for deficiencies.