Abstract
Sclerosing cholangitis is a disease with diffuse stenosis and wall thickness in intra- and extrahepatic biliary tracts. We performed a nationwide survey in Japan of primary sclerosing cholangitis (PSC) and IgG4-related sclerosing cholangitis (IgG4-SC) in 2015, and 428 cases with PSC and 521 cases with IgG4-SC were registered. In PSC, we noticed that there were two peaks in the distribution of the age at presentation, especially in male. IBD was complicated in 39.6% overall but in 69.4% in younger patients. Five-year liver-transplantation free survival rates were 77.0%, and older age, presence of symptoms, and serum albumin at diagnosis were significantly associated with mortality. In IgG4-SC, male to female ratio was 4:1, and 60's were at risk for developing in both male and female. There were 16.1% of patients with serum IgG4 levels within normal range. Type 1 of cholangiographic classification in which stenosis was located at distal biliary tract was the most common. Only three cases were complicated with cholangiocarcinoma. Prednisolone was used in 88.5% of patients and the treatment efficacy was excellent; 5-year survival was 95.2%.
