Title: Treatment of systemic sclerosis with D-penicillamine. A new method of observing the effects of treatment.
Abstract: The pathogenisis of scleroderma is unknown.Histologically it is initially characterized by a gross excess of collagen arranged in irregular bundles, these being later replaced by fibrous tissue and calcification.Harris and Sjoerdsma (1966) have shown the proportion of soluble collagen in this skin to be less than in normal skin.The report by Nimni (1965), showing that the sulphur-containing amino acid penicillamine increases the conversion of insoluble to soluble collagen by disrupting and inhibiting the formation of intermolecular bonds, suggested the possible therapeutic application of this drug in systemic sclerosis (scleroderma).This has been confirmed by Harris and Sjoerdsma (1966), who found a decrease in insoluble and an increase in the poorly cross-linked soluble collagen after short-term administration of penicillamine to patients with scleroderma.By more prolonged dosage there is therefore a possibility of arresting or reversing the widespread tissue sclerosis of this usually slowly progressive disease.We report here the results of a trial of D-penicillamine in eleven patients with systemic sclerosis using subjective and objective criteria of response.