Silica induced scleroderma - Clinical and experimental aspects

Clinical and experimental data concerning silica induced systemic sclerosis (SSc) are discussed in comparison to current knowledge of the pathophysiology of idiopathic SSc. About 280 patients with SSc after longterm silica dust exposure, some with associated silicosis, have been reported; 111 of them were analyzed as the largest cohort in our department. Based on clinical and laboratory data, silica induced and idiopathic SSc show similar pathophysiology and similar markers of the disease including vascular involvement, immunological abnormalities, and dysregulation of extracellular matrix metabolism. Experimental studies show that silica dust is able to activate microvascular endothelial cells, mononuclear cells from peripheral blood, and dermal fibroblasts in vitro in a fashion in common with pathophysiological events known from idiopathic SSc.