[Treatment of systemic scleroderma with factor XIII in 86 patients, with long-term follow-up]. / Traitement de la sclérodermie systémique par le facteur XIII chez 86 malades suivis à long terme.

Eighty-six patients with progressive systemic sclerosis were given coagulation factor XIII intravenously in different dosage regimens. The mean duration of treatment was 19 +/- 18 months and patients were followed up for 22.9 +/- 18.8 months. Improvement or stabilization of the lesions was obtained in 44/86 patients and exclusively concerned skin lesions; there was no improvement in visceral lesions. The drug was well tolerated in short-and long-term treatment. It is concluded that factor XIII demonstrated lasting effectiveness in one-half of the patients treated.

[Evaluation of coagulation factor XIII. Critical study of the different techniques used (author's transl)]. / Evaluation du facteur XIII de la coagulation. Etude critique des techniques utilisées.

A critical study of various techniques of factor XIII determination was performed. The method using dansyl-cadaverin seems to be the most sensitive one but not easily usable in routine tests. The biological techniques using factor XIII-free fibrinogen (screening test and classical test) are satisfactory in clinical trials but are not sensitive for the detection of moderate deficiency of factor XIII. The immunological tests used are the electro-immuno-diffusion test and the test of the consumption of an anti-serum. However, only the antibody directed against the sub unit. A must be used. All these techniques were performed in acquired deficiency of factor XIII and a good correlation was obtained. However, in factor XIII congenital deficiency, we propose to associate a biological assay and the antigenic determination of factor XIII in order to detect a possible abnormal factor XIII.