[New developments in the pathology, pathophysiology and therapy of systemic scleroderma].

In scleroderma, collagen and elastic fibres as well as the dermo-epidermal junction are pathologic. The reactivity of the blood vessels, the function of the smooth muscle cells and the factors protecting against edema formation are defective. The urinary excretion of metabolites of collagen and ground substance glycosaminoglycans indicates quantitatively disease activity. After experimental mass testing in tissue culture, a group of inhibitors of the biosynthesis of essential connective-tissue components were transferred to therapy of scleroderma. In 89 percent of 115 patients, progression could be arrested. After several years of treatment, a regression came about in 74 percent of which 41 percent was subtotal and 17 percent total.