| Theme |
Autoimmune Diseases in Gastroenterology |
| Title |
Primary Sclerosing Cholangitis |
| Author |
Yuji Horiguchi |
Department of Gastroenterology, Fujita Health University School of Medicine |
| Author |
Hideo Imai |
Department of Gastroenterology, Fujita Health University School of Medicine |
| Author |
Hiroshi Sakamoto |
Department of Gastroenterology, Fujita Health University School of Medicine |
| Author |
Tomohiro Suzuki |
Department of Gastroenterology, Fujita Health University School of Medicine |
| [ Summary ] |
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown etiology, characterized by infiltration of inflammatory cells and fibrosis of the intra-and extra-hepatic bile ducts. Four histological stages have been identified. In stage 3, portal-portal fibrous septa and degenerative changes of the bile ducts have progressed, and finally biliary cirrhosis is the result. Liver transplantation (LTx) is the only therapeutic option for patients in the endostage of this disease, whereas medical and/or endoscopic treatments are effective in stage 1-2. The five year survival rate was reported to be more than 80%, although association with bile duct cancer is related to mortality. |