Abstract
Extract: Immunoglobulins (Ig) G, M, and A were significantly elevated in children with focal scleroderma (FS) and progressive systemic scleroderma (PSS), and were significantly higher in those patients who had rheumatoid factor or antinuclear antibodies than in those who did not. Antinuclear antibodies were found in 13 of 23 children with FS and in 5 of 6 children with PSS. Significantly increased binding of antibody to native DNA was detected by radioimmunoassay in 5 of 16 children with FS and in 0 of 6 children with PSS. Persistence of DNA antibody for 6–12 months was demonstrated in two patients with FS and the DNA binding activity correlated with the serum levels of IgG and IgM. The persistence of the high levels of immunoglobulins and autoantibodies are strong evidence of an antigen involved in scleroderma pathogenesis. A case is presented in which antibodies to a specific exogenous antigen (Epstein-Barr virus antigen) were associated with advancing focal scleroderma lesions.
Speculation: The fibrosis of scleroderma is probably initiated by an inflammatory process and is accompanied by significantly increased immune activity which indicates an antigen intimately associated with the disease process. The primary antigen is probably exogenous in origin but may give rise to sclerodermatous changes through secondary effects on local tissue antigens.
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Hanson, V., Kornreich, H., Drexler, E. et al. Some Immunologic Considerations in Focal Scleroderma and Progressive Systemic Sclerosis in Children. Pediatr Res 8, 806–809 (1974). https://doi.org/10.1203/00006450-197409000-00006
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DOI: https://doi.org/10.1203/00006450-197409000-00006
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