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Microvascular changes in progressive systemic sclerosis: immunohistochemical and ultrastructural study.

F Trotta, G Biagini, G Cenacchi

    Clinical and Experimental Rheumatology
    |July 1, 1984
    PubMed
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    This study investigated microvascular changes in Progressive Systemic Sclerosis (PSS). Findings confirm significant microvascular involvement, with swollen endothelial cells and basement membrane thickening in PSS patients.

    Area of Science:

    • Connective tissue diseases
    • Dermatology
    • Immunopathology

    Background:

    • Progressive Systemic Sclerosis (PSS) is a multisystem autoimmune disease.
    • Microvascular abnormalities are increasingly recognized in PSS pathogenesis.

    Purpose of the Study:

    • To investigate the ultrastructural and immunofluorescence characteristics of microvessels in PSS patients.
    • To elucidate the role of endothelial cells and extracellular matrix components in PSS microvasculature.

    Main Methods:

    • Immunofluorescence staining for collagen type IV, laminin, and vimentin.
    • Electron microscopy analysis of dermal microvessels.
    • Evaluation of fibronectin deposition in the deep dermis.

    Main Results:

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    • Immunofluorescence highlighted microvessels with specific antibodies and showed intense vimentin expression in endothelial cells.
    • Electron microscopy revealed swollen endothelial cells occluding microvessels and thickened, laminated basal laminae.
    • Increased fibronectin was observed in the deep dermis.

    Conclusions:

    • The study confirms significant microvascular pathology in PSS, characterized by endothelial cell swelling and basal lamina alterations.
    • These microvascular changes, while not entirely specific, support a crucial role for the vasculature in PSS.
    • Vimentin accumulation within endothelial cells suggests cytoskeletal alterations contributing to microvascular dysfunction.