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Arterial thrombosis in scleroderma.

N L Furey, F R Schmid, H C Kwaan

    The British Journal of Dermatology
    |December 1, 1975
    PubMed
    Summary
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    Scleroderma patients experienced major vessel thrombosis, leading to severe complications like intestinal infarction and limb gangrene. Reduced fibrinolytic activity in affected vessels may contribute to this vascular complication in scleroderma.

    Area of Science:

    • Vascular Biology
    • Rheumatology
    • Pathology

    Background:

    • Scleroderma is a connective tissue disease characterized by fibrosis and vascular abnormalities.
    • Thrombosis of major vessels is a serious, though uncommon, complication of scleroderma.

    Observation:

    • Five patients with scleroderma presented with thrombosis in major arteries.
    • Clinical outcomes included intestinal infarction, limb gangrene, and extremity amputations.
    • Pathological examination revealed severe intimal thickening and thrombus formation in affected arteries.

    Findings:

    • Blood clotting activity was not significantly abnormal in these patients.
    • Histochemical studies indicated reduced fibrinolytic activity at the site of vascular thrombosis.
    • Vascular intimal thickening was a prominent pathological feature.

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    Implications:

    • The vascular system is significantly altered in scleroderma patients.
    • Reduced fibrinolytic activity may be a key factor in scleroderma-associated thrombosis.
    • These findings highlight the critical role of vascular pathology in scleroderma manifestations.