Original StudyScleroderma and IgG4-Related DiseaseReddi, Deepti M. MD*; Cardona, Diana M. MD*; Burchette, James L. HT*; Puri, Puja K. MD*,† Author Information *Department of Pathology, Duke University Medical Center, Durham, NC †Laboratory Corporation of America, Research Triangle Park, NC. Reprints: Puja K. Puri, MD, LabCorp/DIANON, CMBP, T.W. Alexander Dr., RTPl NC 27709 (e-mail: [email protected]). Supported by The Department of Pathology, Duke University. The authors have no conflicts of interest to disclose. The American Journal of Dermatopathology: June 2013 - Volume 35 - Issue 4 - p 458-462 doi: 10.1097/DAD.0b013e318276cbac Buy Metrics Abstract IgG4-related disease is a syndrome which involves lymphoplasmacytic infiltrates and soft tissue sclerosis, elevated serum IgG4 titer, and increased IgG4-positive plasma cells in a variety of tissues. Scleroderma is also characterized by fibrosis and lymphoplasmacytic infiltrates. To our knowledge, the presence of IgG4-positive cells has not been well characterized in scleroderma. A retrospective review of scleroderma and related disorders (calcinosis, raynaud's syndrome, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, progressive systemic sclerosis, morphea) was performed. Thirty-four cases of scleroderma and related disorders were identified; IgG4-positive and IgG-positive plasma cells were counted in 10 HPF and an IgG4:IgG ratio determined. A cutoff ratio of 0.3 was used to define significant elevation. Three of the scleroderma cases had IgG4:IgG greater than 0. Only 1 case had a significant elevation. Of the 3 cases with elevated ratio, IgG4-positive cells ranged from 2 to 64 (median = 14), with an IgG4:IgG ranging from 0.06 to 0.34 (median = 0.22). Similar results were produced with the other sclerosing disorders. These results suggest that scleroderma is not part of the IgG4-related disease spectrum. © 2013 by Lippincott Williams & Wilkins.