DOI 10.5414/CNP72414

Clinical Nephrology, Volume 72 - November (414 - 413)

Treatment of hyper-IgG4 disease with sequential corticosteroids and tamoxifen – case report and review of the literature

S. Chacko1, H. Taskapan2, J. Roscoe3, J. Stein4, E. Woods5, T. Denton6, R. Ting2, 3, P. Tam2, 3, D.G. Oreopoulos7, M. Rodriguez-Justo8, T. Sikaneta2, 3
1 Department of Family Medicine, University of Toronto, Toronto, 2 Nephrology Associates, Scarborough, Ontario, 3 Departments of Nephrology, Medicine, Scarborough General Hospital, 4 Departments of Rheumatology, Medicine, 5 Department of Urology, 6 Diagnostic Imaging, Humber River Regional Hospital, 7 Departments of Nephrology, Medicine, University Health Network, Toronto, Ontario, Canada, 8 Department of Histopathology, Royal Free, University College Medical School, University College Hospital, London, UK

Abstract

We report a patient with multifocal fibrosclerosis presenting as sialadenitis, hepatic fibrosis, and retroperitoneal fibrosis with renal failure. His medical management consisted of prednisone (4 months at 40 mg daily, then tapered down to 5 mg daily for another 14 months) and 18 months of tamoxifen. He responded clinically and radiographically to this regimen, and remains in clinical remission 10 months after discontinuing medical therapy. Subsequent histologic examination of submandibular gland tissue revealed strong staining for IgG4-positive plasma cells. To our knowledge, this is the first case of confirmed multifocal hyper-IgG4 disease to be successfully treated with sequential corticosteroids and tamoxifen.

Author Details

Authors

  • S. Chacko1
  • H. Taskapan2
  • J. Roscoe3
  • J. Stein4
  • E. Woods5
  • T. Denton6
  • R. Ting2
  • 3
  • P. Tam2
  • 3
  • D.G. Oreopoulos7
  • M. Rodriguez-Justo8
  • T. Sikaneta2
  • 3

Departments

  • 1 Department of Family Medicine, University of Toronto, Toronto,
  • 2 Nephrology Associates, Scarborough, Ontario,
  • 3 Departments of Nephrology, Medicine, Scarborough General Hospital,
  • 4 Departments of Rheumatology, Medicine,
  • 5 Department of Urology,
  • 6 Diagnostic Imaging, Humber River Regional Hospital,
  • 7 Departments of Nephrology, Medicine, University Health Network, Toronto, Ontario, Canada,
  • 8 Department of Histopathology, Royal Free, University College Medical School, University College Hospital, London, UK

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