SILICA-INDUCED SCLERODERMA - CLINICAL AND EXPERIMENTAL ASPECTS

Clinical and experimental data concerning silica induced systemic scle rosis (SSc) are discussed in comparison to current knowledge of the pa thophysiology of idiopathic SSc. About 280 patients with SSc after lon gterm silica dust exposure, some with associated silicosis, have been reported; 111 of them were analyzed as the largest cohort in our depar tment. Based on clinical and laboratory data, silica induced and idiop athic SSc show similar pathophysiology and similar markers of the dise ase including vascular involvement, immunological abnormalities, and d ysregulation of extracellular matrix metabolism. Experimental studies show that silica dust is able to activate microvascular endothelial ce lls, mononuclear cells from peripheral blood, and dermal fibroblasts i n vitro in a fashion in common with pathophysiological events known fr om idiopathic SSc.