Immunoglobulin G4-related disease (IgG4-RD) represents a group of disorders that share features of inflammation, plasma cell infiltrates, and fibrosis. Sclerosing cholangitis is a disorder involving inflammation, scarring, and destruction of the bile ducts. IgG4-related sclerosing cholangitis (IgG4-SC) has been proposed as a bile duct lesion associated with IgG4-RD. This disease entity can be distinguished from other types of sclerosing cholangitis and classified into four types based upon the region of strictures revealed by cholangiography. Here, we present two cases in which the finding of bile duct wall thickening visualized with transabdominal ultrasonography was useful in the diagnosis of patients with IgG4-SC. At present, transabdominal ultrasonography is not included in the diagnostic algorithm for IgG4-SC. We are certain that detailed observation of the bile duct wall with transabdominal ultrasonography can make a significant contribution to the diagnosis of IgG4-SC. Furthermore, we propose that transabdominal ultrasonography may be useful in following clinical improvement in cases where a steroid trial is the best option for treatment. Both cases emphasize the practicality of transabdominal ultrasonography in the diagnosis and follow-up observation of IgG4-SC.
Keywords: IgG4; IgG4-related disease; IgG4-related sclerosing cholangitis; Immunoglobulin; Sclerosing cholangitis; Transabdominal ultrasonography.