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Juvenile dermatomyositis and polymyositis.

L M Pachman, M C Maryjowski

    Clinics in Rheumatic Diseases
    |April 1, 1984
    PubMed
    Summary

    Juvenile dermatomyositis (JDMS) is a distinct childhood autoimmune disease. Steroid treatment significantly reduces mortality, but calcifications remain a debilitating complication requiring further research.

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    Decreased levels of CD54 (ICAM-1)-positive lymphocytes in the peripheral blood in untreated patients with active juvenile dermatomyositis.

    Clinical and diagnostic laboratory immunology·2000

    Area of Science:

    • Pediatrics
    • Rheumatology
    • Immunology

    Background:

    • Childhood myositis presents with muscle enzyme elevation, weakness, and inflammation.
    • Juvenile dermatomyositis (JDMS) is more common than polymyositis (PM) in children, particularly females.
    • Steroid therapy has decreased JDMS mortality from 33% to 7%.

    Purpose of the Study:

    • To establish Juvenile Dermatomyositis (JDMS) as a distinct disease entity.
    • To explore the immunogenetic associations and immunological abnormalities in JDMS.
    • To investigate the potential role of viral infections and endothelial cell pathology in JDMS pathogenesis.

    Main Methods:

    • Review of clinical characteristics, treatment outcomes, and mortality rates in JDMS.
    • Analysis of HLA associations (HLA-B8, -DR3) and immunological markers (natural killer cell activity, complement activation, ANA).
    • Investigation of viral antibodies (Coxsackie B) and pathological findings (endothelial cell inclusions, Factor VIII levels).

    Main Results:

    • JDMS is hypothesized as a distinct immunopathic disorder, with increased HLA-B8 and -DR3.
    • Immunological abnormalities include impaired natural killing and complement activation; positive ANA is frequent.
    • Pathological findings show endothelial cell inclusions linked to vessel occlusion and elevated Factor VIII in active disease.

    Conclusions:

    • JDMS is proposed as a distinct disease entity with immunopathic features.
    • Calcifications are a significant debilitating complication (33% of cases).
    • Further evaluation of immunosuppressive agents and plasmapheresis in severe JDMS is warranted.

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