| Theme | Autoimmune Pancreatitis | |
|---|---|---|
| Title | Sclerosing Cholangitis Associatedwith Autoimmune Pancreatitis (IgG4-relatedSclerosing Cholangitis) | |
| Publish Date | 2010/08 | |
| Author | Itaru Naitoh | Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences |
| Author | Hirotaka Ohara | Division of General Medicine, Nagoya City University Hospital |
| Author | Takahiro Nakazawa | Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences |
| Author | Tomoaki Ando | Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences |
| Author | Kazuki Hayashi | Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences |
| Author | Takashi Joh | Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences |
| [ Summary ] | Autoimmune pancreatitis (AIP) is frequently associated with sclerosing cholangitis termed IgG4-related sclerosing cholangitis (IgG4-SC). Primary sclerosing cholangitis (PSC) and cholangiocarcinoma (CC) are important diseases with differing diagnoses because these diseases are different from IgG4-SC in both means of treatment and prognosis. IgG4-SC usually responds well to steroid therapy. In contrast,liver transplantation is the only effective therapy for PSC and surgical procedures are required for chlangiocarcinoma. High serum IgG4 values,coexistence with AIP and other organ involvement are useful factors for diagnosing IgG4-SC. The association with right-dominant inflammatory bowel syndrome suggests PSC. Cholangiograms are useful for discriminating IgG4-SC from PSC. Band-like strictures,beaded appearance and pruned tree appearance are characteristicly seen in cholangiograms for PSC. Segmental strictures,long strictures with prestenotic dilation and lower common bile duct strictures are characteristic findings associated with IgG4-SC. Intraductal ultrasonograpy(IDUS)findings in the regions of strictures and non-strictured regions as observed in cholangiograms are useful to discriminate IgG4-SC from CC. The most characteristic IDUS finding of IgG4-SC is wall thickening in non-strictured regions,as observed in cholangiograms which discriminate it from CC. Wall thickness spreads from the intrapancreatic bile duct to the upper bile duct continuously in most IgG4-SC cases. | |