Treatment of generalized scleroderma with inhibitors of connective tissue formation.

103 patients suffering from generalized scleroderma were studied in order to assess the effect of treatment with inhibitors of connective tissue formation. 93 patients with generalized scleroderma were given D-penicillamine, benzyl-penicillin-diethylamino-ethyl-ester hydroiodide, adrenal glucocorticoids, dextro-thyroxine, hydralazine, and "mixed treatment" (one or several of the drugs in consecutive courses, or concurrently). The effect of dextrothyroxine could not be evaluated in this study. No improvement could be seen after adrenocortical steroid therapy. Hydralazine seemed to be effective. D-penicillamine improved 25 of 34 treated patients; penicillin hydroiodide 12 out of 16. The dermal sclerosis of 6 patients regressed completely; in 16, sclerosis regressed with the exception of finger sclerosis; in 32, partial regression was registered; 20 had their progression arrested, but there was no regression; in 19 cases, there was no effect whatsoever. The prognosis seemed to be better for young than for old people. The age at onset was lower in the better groups. The higher the total dose, the better the results. The length of the treatment course is probably of some significance. The short-lasting cases had better prospects than the longer lasting. Ten untreated patients of this material and 11 patients seen earlier showed continued progression. Side effects leading to discontinuation of the drugs were seen in a substantial number of patients, especially after D-penicillamine. Twelve deaths could not be related to the treatments.