Scleroderma and IgG4-related disease

Am J Dermatopathol. 2013 Jun;35(4):458-62. doi: 10.1097/DAD.0b013e318276cbac.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers / analysis
  • Biopsy
  • Female
  • Fibrosis
  • Humans
  • Immunoglobulin G / analysis*
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Plasma Cells / immunology*
  • Retrospective Studies
  • Scleroderma, Systemic / immunology*
  • Scleroderma, Systemic / pathology
  • Skin / immunology*
  • Skin / pathology
  • Young Adult


  • Biomarkers
  • Immunoglobulin G