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

  • Radiol Clin North Am. 2016 May;54(3):535-51. doi: 10.1016/j.rcl.2015.12.010.
Venkata S Katabathina  1 ,  Suhare Khalil  2 ,  Sooyoung Shin  3 ,  Narayan Lath  4 ,  Christine O Menias  5 ,  Srinivasa R Prasad  3
Affiliations
  • 1. Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA. Electronic address: [email protected].
  • 2. Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA.
  • 3. Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
  • 4. Department of Radiology, Singapore General Hospital, Outram road, Singapore 169608, Singapore.
  • 5. Department of Radiology, Mayo Clinic, Scottsdale, AZ, USA.
PMID: 27153787 DOI: 10.1016/j.rcl.2015.12.010
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.

Keywords
18FDG-PET; Autoimmune pancreatitis; Computed tomography; IgG4 cholangitis; IgG4-related disease; Lymphoplasmacytic infiltration; Magnetic resonance imaging; Riedel thyroiditis.