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Immunological studies in childhood scleroderma.

W Stögmann, M Sandhofer, J Fritz

    European Journal of Pediatrics
    |February 21, 1977
    PubMed
    Summary
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    Immunological studies in children with scleroderma reveal significant autoimmunity, including antinuclear and DNA antibodies. Some patients also showed immune deficiency, suggesting it may predispose to progressive systemic sclerosis.

    Area of Science:

    • Immunology
    • Rheumatology
    • Pediatrics

    Background:

    • Scleroderma encompasses systemic sclerosis (PSS) and focal forms like scleroderma en bandes.
    • Autoimmunity is implicated in autoimmune connective tissue diseases.
    • Pediatric scleroderma requires further immunological investigation.

    Purpose of the Study:

    • To investigate the immunological profiles of children with scleroderma.
    • To determine the presence of autoantibodies and immune cell abnormalities.
    • To explore the role of autoimmunity and immune deficiency in pediatric scleroderma.

    Main Methods:

    • Immunological studies were conducted on 13 children diagnosed with scleroderma.
    • Analysis included detection of antinuclear antibodies with a speckled fluorescence pattern.

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  • Flow cytometry was used to assess T cell and B cell percentages.

    • Main Results:

    • Antinuclear antibodies with speckled fluorescence were detected in patients with systemic sclerosis and scleroderma en bandes.
    • High levels of DNA antibodies were found in most patients with systemic sclerosis and scleroderma en bandes.
    • Two patients with progressive systemic sclerosis exhibited a low percentage of T cells and an increased percentage of B cells.

    Conclusions:

    • Autoimmunity plays a significant role in both systemic sclerosis and focal scleroderma in children.
    • Immune deficiency, particularly a defect in cellular immunity, may predispose to progressive systemic sclerosis.
    • These findings highlight the importance of immunological assessment in pediatric scleroderma management.