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Case Reports in Rheumatology
Volume 2017 (2017), Article ID 4716245, 4 pages
Case Report

Thoracic Paravertebral Mass as an Infrequent Manifestation of IgG4-Related Disease

1Division of Rheumatology, Detroit Medical Center, Wayne State University, Henry Ford Hospital, Detroit, MI, USA
2Henry Ford Medical Group, Detroit, MI, USA
3Division of Rheumatology, Henry Ford Medical Group, Detroit, MI, USA

Correspondence should be addressed to Melissa Matzumura Kuan

Received 17 June 2017; Accepted 27 August 2017; Published 28 December 2017

Academic Editor: George S. Habib

Copyright © 2017 Melissa Matzumura Kuan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Case. A 50-year-old African American male presented with abdominal pain and significant weight loss. On physical examination, he had parotid and submandibular gland enlargement associated with right eye proptosis. Computed tomography showed a thoracic paravertebral soft tissue mass, enlarged lymph nodes, and ascending aortic aneurysm. Laboratory results were remarkable for elevated total IgG and IgG4 subclass. The submandibular gland pathology revealed chronic sclerosing sialadenitis, with a large subset of inflammatory cells positively staining for IgG4. The histology of the paravertebral mass demonstrated fibrosclerosis with increased lymphocytic infiltrate, associated with increased IgG4 plasma cells. He was diagnosed with immunoglobulin G4-related disease (IgG4-RD). Steroid therapy initially yielded improvement; however, after steroids were stopped, there was disease recurrence. Prednisone was restarted, and the plan was to start him on rituximab. Interestingly, the patient’s brother also had IgG4-RD. Conclusion. IgG4-RD can present as a paravertebral mass which is usually responsive to steroids; however, recurrent and resistant disease can be seen for which steroid-sparing agents such as rituximab should be considered. In addition, to the best of our knowledge, this is the first reported case of IgG4-RD in two family members presenting as a paravertebral mass, highlighting an exciting area for more research in the future.