| [ Summary ] |
Primary sclerosing cholangitis (PSC) is an uncommon disease characterized by chronic cholestasis caused by diffuse inflammation and fibrosis that involves the entire biliary tract. In Japan, patients with PSC are clinically classified into the two groups, typical and non-typical cases. The disease is progressive in the typical cases. On the other hand, the cholangiographic findings and liver function tests improve with treatment (biliary drainage and/or drug administration) in the non-typical cases which show the transient obstructive jaundice. Ages of onset in the typical cases were significantly younger than in the non-typical cases. Clinical features appear to differ between these two groups. Most younger patients with PSC are typical cases and the disease is progressive. However, patients with a favorable prognosis are observed among the elderly. It is likely that there are several clinical features in these patients. |