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Scleroderma: developments from Osler to the present

P H Cunningham, B S Andrews, J S Davis

    Southern Medical Journal
    |June 1, 1980
    PubMed
    Summary
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    Progressive systemic sclerosis (PSS), or scleroderma, remains a clinical mystery. Research explores collagen, immunity, and vascular factors, but the cause of PSS is still unknown.

    Area of Science:

    • Rheumatology
    • Immunology
    • Vascular Biology

    Background:

    • Progressive systemic sclerosis (PSS), also known as scleroderma, has a clinical definition unchanged since 1892.
    • Related and overlap syndromes offer potential insights into PSS etiology.
    • The fundamental cause of PSS remains elusive despite extensive research.

    Purpose of the Study:

    • To review current research areas concerning the etiology of PSS.
    • To highlight the ongoing investigation into collagen synthesis, immune system roles, and vascular complications.
    • To assess the limited therapeutic advances for PSS.

    Main Methods:

    • Literature review of PSS research.
    • Analysis of etiological factors including collagen abnormalities, cellular and humoral immunity.

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  • Examination of the relationship between PSS, vascular disease, and hyperreactivity.

    • Main Results:

    • Current research focuses on collagen synthesis, immune system involvement (cellular and humoral), and vascular disease in PSS.
    • No definitive cause for PSS has been identified.
    • Therapeutic progress has been minimal, with notable exceptions in managing malignant hypertension and renal failure.

    Conclusions:

    • The etiology of PSS remains a significant research challenge.
    • Understanding immune and vascular components is crucial for future research.
    • Effective treatments for the systemic aspects of PSS are still limited.