[Update on the treatment of systemic scleroderma].
Renewed clinical trials of drugs used for many years and the availability of new symptomatic therapies allows selection of appropriate treatment for progressive systemic sclerosis. Colchicine damages intracytoplasmic microtubules and when administered at a dose of at least 1.5 mg per day during early stages of the disease reduces cutaneous sclerosis without improving other manifestations of scleroderma. D-penicillamine inhibits bridge formation during collagen maturation, acting mainly on cutaneous infiltration with more uncertain effects on visceral localizations of the disease. It can be effective against pulmonary lesions but, as with colchicine, must be prescribed early. Its action is delayed and its side effects limit its use. Corticoids are of very limited efficiency and although they may be useful for relief of muscular and articular localizations they have been accused of precipitating onset of renal insufficiency. Immunodepressants have until now been assessed as ineffective. The demonstration in patients with progressive systemic sclerosis of abnormal activation of immunity system cells suggested the use in this collagen disease of a new immunosuppressive agent, cyclosporine A, but clinical utility of this drug remains to be demonstrated. In systemic sclerosis efficacy of plasma exchange is mainly directed against vasomotor disorders and digital ulcerations, but study results are difficult to assess because of associated therapies. Constraints and risks of this treatment also considerably reduce its interest. The coagulation factor XIII acts as a stabilizing factor of collagen. It reduces cutaneous infiltration but its efficacy is based on results of a single controlled trial and its use is limited by the need for repeated intravenous injections.(ABSTRACT TRUNCATED AT 250 WORDS)
Priollet
P
P
Cazalis
P
P
Valleteau de Moulliac
M
M
D006801:Humans; D012595:Scleroderma, Systemic / Q000628:therapy*
Actualités dans le traitement de la sclérodermie systémique.