Primary sclerosing cholangitis overview
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Differentiating Primary sclerosing cholangitis from other Diseases |
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Diagnosis |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dima Nimri, M.D. [2]
Overview
Historical Perspective
Due to the rarity of the disease, primary sclerosing cholangitis has not been well studied in the past and cases were reported as individual cases. The first case of primary sclerosing cholangitis was documented in 1924.
Classification
Primary sclerosing cholangitis may be classified according to the size of bile ducts involved, as well as its association with autoimmune hepatitis. The 3 main subtypes are the classic subtype, small ducts subtype and primary sclerosing cholangitis associated with autoimmune hepatitis.
Pathophysiology
Causes
The cause of primary sclerosing cholangitis is unclear and not fully understood. However, genetic and environmental factors are thought to contribute to the development of the disease.
Differentiating primary sclerosing cholangitis from Other Diseases
Primary sclerosing cholangitis must be differentiated from causes of secondary sclerosing cholangitis.
Epidemiology and Demographics
Primary sclerosing cholangitis is a relatively rare disease, with the incidence ranging from 0 to 1.3 per 100,000 cases and prevalence of 0 to 16.2 per 100,000 cases. The disease primarily affects young adult males and the mean age at diagnosis is 41 years.