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Using En Face Immunofluorescence Staining to Observe Vascular Endothelial Cells Directly
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Factor XIII and Endothelial Dysfunction in Patients with Systemic Sclerosis.

Sonja Alesci1,2, Matthias Wahle3, Andrea Himsel4

  • 1Frankfurt Haemophilia Centre, Goethe University Frankfurt, Frankfurt am Main, Hessen, Germany.

Hamostaseologie
|May 1, 2023
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Summary

Coagulation factor XIII (FXIII) may indirectly improve systemic sclerosis (SSc) skin symptoms by moderating endothelial dysfunction. This study found no FXIII deficiency in SSc patients but noted correlations between von Willebrand factor antigen and lung issues and skin severity.

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Area of Science:

  • Rheumatology
  • Hematology
  • Immunology

Background:

  • Systemic sclerosis (SSc) is a severe autoimmune disease impacting skin and organs.
  • Coagulation factor XIII (FXIII) shows potential benefits in SSc patient outcomes.
  • Investigating FXIII's role alongside endothelial dysfunction and skin issues in SSc is crucial.

Purpose of the Study:

  • To explore the relationship between coagulation FXIII, endothelial dysfunction markers, and skin manifestations in SSc patients.
  • To assess correlations between FXIII activity, modified Rodnan skin scores (MRSS), and daily activity limitations (SHAQ).
  • To examine the association of FXIII with Raynaud's phenomenon and pain levels.

Main Methods:

  • A single-center cohort study included 56 SSc patients.
  • Analyzed markers of inflammation, coagulation (including FXIII, FVIII, VWF-Ag, D-dimers), and endothelial dysfunction (VWF-Ag, VEGF).
  • Evaluated modified Rodnan skin scores (MRSS) and Scleroderma Health Assessment Questionnaire (SHAQ) for skin involvement and daily activities.

Main Results:

  • No significant correlation was found between FXIII activity, MRSS, and SHAQ scores.
  • FXIII activity correlated with Raynaud's phenomenon symptoms and weakly with pain.
  • A significant correlation between VWF-Ag and lung complaints (p<0.0001) and between VWF-Ag and MRSS (p=0.01) was observed, indicating more severe skin involvement with higher VWF-Ag.
  • A trend towards a negative correlation between FXIII activity and VWF-Ag was noted (p=0.15).

Conclusions:

  • No FXIII deficiency was identified in the studied SSc cohort.
  • FXIII may play an indirect role in improving SSc cutaneous manifestations by influencing endothelial dysfunction.
  • Further clinical research is necessary to confirm FXIII's role in SSc management.