[Therapeutic action of coagulation factor XIII in scleroderma. 20 cases].
Clotting factor XIII was used in 13 patients suffering from scleroderma, 17 with generalised scleroderma and 3 with localised scleroderma. The duration of treatment varied between 15 days and 6 months. The effect was marked in 7 cases and more moderate in 5 others. It took the form of increased suppleness of the skin, improved joint mobility and, to a lesser degree, an improvement in vasomotor disturbances of the extremities. Oesophageal abnormalities on X-ray were never altered. The return to normal of alveolar-capillary diffusion seen in two cases requires futher confirmation. The action of factor XIII in scleroderma may be explicable by the formation of transamidation bonds between the alpha chains of the collagen molecule, similar to those obtained on fibrin.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002986:Clinical Trials as Topic; D003094:Collagen / Q000378:metabolism; D005176:Factor XIII / Q000378:metabolism / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011487:Protein Conformation; D011653:Pulmonary Diffusing Capacity / Q000187:drug effects; D011928:Raynaud Disease / Q000628:therapy; D012008:Recurrence; D012594:Scleroderma, Localized / Q000188:drug therapy*; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D012867:Skin / Q000187:drug effects; D014797:Vital Capacity / Q000187:drug effects
D003094:Collagen; D005176:Factor XIII
Action thérapeutique du facteur XIII de la coagulation dans la sclérodermie. 20 cas