title: 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.
authors.affiliation: Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA. Electronic address: katabathina@uthscsa.edu.
authors.forename: Venkata S
authors.initials: VS
authors.lastname: Katabathina
authors.affiliation: Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA.
authors.forename: Suhare
authors.initials: S
authors.lastname: Khalil
authors.affiliation: Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Sooyoung
authors.initials: S
authors.lastname: Shin
authors.affiliation: Department of Radiology, Singapore General Hospital, Outram road, Singapore 169608, Singapore.
authors.forename: Narayan
authors.initials: N
authors.lastname: Lath
authors.affiliation: Department of Radiology, Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Christine O
authors.initials: CO
authors.lastname: Menias
authors.affiliation: Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Srinivasa R
authors.initials: SR
authors.lastname: Prasad
mesh_terms: D001327:Autoimmune Diseases / Q000000981:diagnostic imaging* / Q000276:immunology*; D003937:Diagnosis, Differential; D019317:Evidence-Based Medicine; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007249:Inflammation / Q000000981:diagnostic imaging* / Q000276:immunology*; D008279:Magnetic Resonance Imaging / Q000379:methods; D009102:Multiple Organ Failure / Q000000981:diagnostic imaging* / Q000276:immunology; D049268:Positron-Emission Tomography / Q000379:methods; D019275:Radiopharmaceuticals; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D007074:Immunoglobulin G; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18
keywords: 18FDG-PET; Autoimmune pancreatitis; Computed tomography; IgG4 cholangitis; IgG4-related disease; Lymphoplasmacytic infiltration; Magnetic resonance imaging; Riedel thyroiditis