Scleroderma and IgG4-Related Disease

IgG(4)-related disease is a syndrome which involves lymphoplasmacytic infiltrates and soft tissue sclerosis, elevated serum IgG(4) titer, and increased IgG(4)-positive plasma cells in a variety of tissues. Scleroderma is also characterized by fibrosis and lymphoplasmacytic infiltrates. To our knowledge, the presence of IgG(4)-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; IgG(4)-positive and IgG-positive plasma cells were counted in 10 HPF and an IgG(4):IgG ratio determined. A cutoff ratio of 0.3 was used to define significant elevation. Three of the scleroderma cases had IgG(4):IgG greater than 0. Only 1 case had a significant elevation. Of the 3 cases with elevated ratio, IgG(4)-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 IgG(4)-related disease spectrum.