| Theme | PSC and PBC -- Recent Progress in Diagnosis and Treatment | |
|---|---|---|
| Title | Differentiation between PSC and IgG4-related Sclerosing Cholangitis | |
| Publish Date | 2014/10 | |
| Author | Terumi Kamisawa | Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, |
| Author | Sawako Kuruma | Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, |
| Author | Taku Tabata | Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, |
| Author | Susumu Iwasaki | Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, |
| Author | Kazuro Chiba | Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, |
| Author | Satomi Koizumi | Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, |
| [ Summary ] | Primary sclerosing cholangitis (PSC) involves the small and large intrahepatic bile ducts, as well as the extrahepatic bile duct. The epithelium of the bile duct is often damaged. Also IgG4-related sclerosing cholangitis (SC) involves the large bile duct, with the epithelium usually being preserved. Abundant infiltration of IgG4-positive plasma cells observed in liver biopsies and bile duct biopsies along with major papilla highly suggests IgG4-SC. This condition occurs predominantly in elderly males, and is frequently associated with other IgG4-related diseases such as autoimmune pancreatitis. Elevation of serum IgG4 levels is frequently detected in IgG4-SC patients. A beaded and pruned-tree appearance is frequently detected with cholangiography of PSC patients. Stenosis of the lower bile duct and long strictures in the hilar bile duct are characteristic cholangiographic findings for patients with IgG4-SC. Since IgG4-SC responds well to steroid administration, the effectiveness of steroid therapy is an optional diagnostic mode for IgG4-SC. | |