| Theme |
Development of Diagnosis and Therapy for Biliary Tract Diseases |
| Title |
Differential Diagnosis between Primary Sclerosing Cholangitis and IgG4-related Sclerosing Cholangitis |
| Author |
Terumi Kamisawa |
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital |
| Author |
Kensuke Takuma |
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital |
| Author |
Taku Tabata |
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital |
| Author |
Yoshihiko Inaba |
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital |
| Author |
Naoto Egawa |
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital |
| [ Summary ] |
Sclerosing cholangitis is a heterogeneous disease including primary sclerosing cholangitis (PSC), secondary sclerosing cholangitis, and IgG4-related sclerosing cholangitis. PSC is a progressive disease of unknown origin, and involves the intra and extrahepatic bile ducts, sometimes resulting in liver cirrhosis. PSC is sometimes associated with inflammatory bowel disease and cholangiocarcinoma.
IgG4-related sclerosing cholangitis occurs frequently in elderly males with autoimmune pancreatitis and other sclerosing diseases, and responds well to steroid therapy. Elevation of serum IgG4 levels and dense infiltration of IgG4-positive plasma cells are characteristic in IgG4-related sclerosing cholangitis. While 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-related sclerosing cholangitis. PSC and IgG4-related sclerosing cholangitis are distinctly different disease entities, and the two diseases should be correctly diagnosed and treated. |