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)