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Coagulation factor XIII in scleroderma

D Jullien1, A L Souillet, M Faure

  • 1Clinique Dermatologique, Hôpital E.-Herriot/Hospices Civils de Lyon, 69437 Lyon Cedex 03, France. INSERM U.98, Faculté Laennec, 69372 Lyon Cedex 08, France. julien@laennec.univ-lyon1.fr

European Journal of Dermatology : EJD
|July 3, 1998
PubMed
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Blood coagulation factor XIII (FXIII) showed promise in treating scleroderma by improving skin and musculoskeletal symptoms. Reconsidering FXIII therapy for scleroderma is suggested due to safer concentrate availability.

Area of Science:

  • Rheumatology
  • Hematology
  • Dermatology

Background:

  • Blood coagulation factor XIII (FXIII) influences collagen metabolism, suggesting a role in fibrotic diseases like scleroderma.
  • FXIII was previously investigated for scleroderma treatment, yielding positive initial outcomes for skin sclerosis and musculoskeletal symptoms.

Purpose of the Study:

  • To review existing data on FXIII use in scleroderma.
  • To propose prescribing guidelines for FXIII in scleroderma.
  • To stimulate debate on expanding FXIII's regulated use to scleroderma treatment.

Main Methods:

  • Review of available literature on FXIII and scleroderma.
  • Analysis of historical treatment outcomes.
  • Formulation of proposed prescribing principles.

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Main Results:

  • Initial studies indicated FXIII effectively treated skin sclerosis, musculoskeletal involvement, and weakness in scleroderma patients.
  • Further clinical assessment was halted due to regulatory restrictions on FXIII following the HIV epidemic.

Conclusions:

  • Safer FXIII concentrates are now available, warranting a re-evaluation of its therapeutic potential in scleroderma.
  • There is a need to consider widening the regulated use of FXIII for scleroderma management.