S. Chacko¹, H. Taskapan², J. Roscoe³, J. Stein⁴, E. Woods⁵, T. Denton⁶, R. Ting²,³, P. Tam²,³, D.G. Oreopoulos⁷, M. Rodriguez-Justo⁸ and T. Sikaneta²,^{3 1}Department of Family Medicine, University of Toronto, Toronto, ²Nephrology Associates, Scarborough, Ontario, ³Departments of Nephrology and Medicine, Scarborough General Hospital, ⁴Departments of Rheumatology and Medicine, ⁵Department of Urology, ⁶Diagnostic Imaging, Humber River Regional Hospital, ⁷Departments of Nephrology and Medicine, University Health Network, Toronto, Ontario, Canada, and ⁸Department of Histopathology, Royal Free and University College Medical School, University College Hospital, London, UK 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.Correspondence to:
Dr. T. Sikaneta
Nephrology Associates
Scarborough, Ontario, M1H 3G4, Canada
Email: tabosikaneta@yahoo.ca