| [ Summary ] |
PSC is a progressive disease associated with fibrous strictures in the intra- and extrahepatic bile ducts. There are two peaks in the age distribution of PSC in Japan. The younger patients frequently have complications such as inflammatory bowel disease (IBD). Sclerosing cholangitis complicated with autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis is considered to be a different entity than PSC. Specific findings associated with the bile duct, such as beaded appearance, are most important for diagnosing PSC. Jaundice, itching and increased ALP activity are often observed with PSC, but not in all cases. Medications and endoscopic therapies may be employed, but are sometimes not effective. Some of these cases may be candidates for liver transplantation. |