PARALLEL SYMPOSIUM 2 : Primary Sclerosing Cholangitis and IgG4-Associated Cholangitis = PARALLEL SYMPOSIUM 2 : Primary Sclerosing Cholangitis and IgG4-Associated Cholangitis

다국어 초록 (Multilingual Abstract)

Primary sclerosing cholangitis (PSC) is a chronic and progressive disorder characterized by inflammation and fibrosis of intra- and extrahepatic bile ducts which finally leads to biliary cirrhosis. PSC is seen as immune-mediated disorder but the pathogenesis is still unclear. PSC has a strong association with inflammatory bowel disease (IBD). In addition, PSC patients are at high risk to develop cholangiocarcinoma, colorectal cancer, gallbladder cancer, and hepatocellular carcinoma. Therefore, the surveillance for the development of various cancers is important. The prognosis is poor and the median survival is in the range of 11 to 18 years. Pharmacologic treatment does not prolong survival. The dominant biliary strictures need endoscopic balloon dilatation and/or stenting. Liver transplantation is recommended in patients with end-stage liver disease, intractable pruritus, recurrent bacterial cholangitis. IgG4-associated cholangitis (IAC) is a recently defined disease entity which shares a number of clinical, laboratory, and radiological features with PSC. However, patients with IAC are older, present more abruptly with obstructive jaundice, and not associated with IBD. IAC is one entity of IgG4-associated systemic sclerosing disorder which is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs including pancreas, bile duct, salivary glands, kidney, prostate, retroperitoneum, and lymph nodes. Most patients respond to steroid therapy but disease can be relapsed.