Immunoglobulin G4-Related Disease: Recent Advances in Pathogenesis and Imaging Findings

Abstract Immunoglobulin G4-related disease (IgG4-RD) is a novel, immune-mediated, multisystem disease characterized by the development of tumefactive lesions in multiple organs. IgG4-RD encompasses many fibroinflammatory diseases that had been thought to be confined to single organs. Delayed diagnosis or misdiagnosis as malignancies leading to aggressive treatment may be averted by identification of the multisystem nature of IgG4-RD. Most cases show exquisite response to steroid therapy; steroid-resistant cases are being treated by novel therapeutic agents, including B-cell depleting agents such as rituximab. Cross-sectional imaging studies play a pivotal role in the initial diagnosis, assessing response to therapy and long-term surveillance.