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Coagulation factor XIII, endothelial damage and systemic sclerosis.

A V Marzano1, A B Federici, G Gasparini

  • 1Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore, Milan, Italy.

European Journal of Dermatology : EJD
|February 29, 2000
PubMed
Summary
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Factor XIII (F XIII) may improve endothelial damage in systemic sclerosis (SSc). Studies show F XIII treatment lowered von Willebrand factor antigen (vWF:Ag) levels, a marker of endothelial injury, in SSc patients.

Area of Science:

  • Hematology
  • Rheumatology
  • Vascular Biology

Background:

  • Factor XIII (F XIII) stabilizes fibrin clots and is investigated for scleroderma treatment.
  • Its precise mechanism, particularly regarding endothelial function in systemic sclerosis (SSc), is not fully understood.

Purpose of the Study:

  • To investigate the potential effects of F XIII on endothelial damage in patients with systemic sclerosis (SSc).
  • To assess F XIII's role in the early pathogenic events of SSc.

Main Methods:

  • Measured plasma levels of von Willebrand factor antigen (vWF:Ag) as a marker of endothelial injury.
  • Analyzed F XIII plasma concentrations in 22 SSc patients (9 treated with F XIII, 13 untreated).

Main Results:

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  • SSc patients treated with F XIII showed significantly lower vWF:Ag levels compared to untreated patients (p < 0.02).
  • Elevated vWF:Ag levels correlated with disease severity, particularly in patients with severe lung involvement.
  • F XIII plasma concentrations were normal in most SSc patients, excluding deficiency as a cause.

    • Conclusions:

    • Preliminary findings suggest F XIII may ameliorate endothelial damage in SSc.
    • This potential benefit extends beyond F XIII's known effects on collagen metabolism.