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

Authors:
Venkata S Katabathina
Venkata S Katabathina
University of Texas Health Science Center at San Antonio
United States
Suhare Khalil
Suhare Khalil
University of Texas Health Science Center at San Antonio
San Antonio | United States
Sooyoung Shin
Sooyoung Shin
Ajou University College of Pharmacy
Christine O Menias
Christine O Menias
Mallinckrodt Institute of Radiology
United States
Srinivasa R Prasad
Srinivasa R Prasad
University of Texas Health Science Center at San Antonio
United States

Radiol Clin North Am 2016 May;54(3):535-51

Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

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.

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http://dx.doi.org/10.1016/j.rcl.2015.12.010DOI Listing

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May 2016
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