Recent progress of lgG4-related hepatobiliary diseases with emphasis on pathologic aspects and differential diagnosis

摘要

lgG4-related disease usually presents with a mass-forming or regional lesion showing considerable lymphoplasmacytic infiltration with many lgG4-positive plasma cells, sclerosis, and obliterative phlebitis. Herein, recent progress of hepatobiliary lgG4-related diseases including lgG4-related sclerosing cholangitis (lgG4-SC), hepatic inflammatory pseudotu-mor (HIP), and autoimmune hepatitis (AIH), are reviewed. lgG4-SC mainly affects the extrahepatic and hilar bile ducts, and is almost always associated with autoimmune pancreatitis (AIP), a prototype of lgG4-related disease. lgG4-SC has a propensity to exaggerate focally, and such lgG4-SC could be categorized as an lgG4-related HIP, mainly belonging to the lymphoplasmacytic type of HIP. lgG4-related AIH is a chronic active hepatitis with many lgG4-positive plasma cells and increased serum lgG4 level. In conclusion, several hepatobiliary diseases which had been diagnosed as other diseases, can be reclassified as lgG4-related diseases, and several local factors peculiar to the hepatobiliary system may be responsible for the development of these diseases.