Haustein U F, Anderegg U
Department of Dermatology, School of Medicine, Leipzig University, Germany.
J Rheumatol. 1998 Oct;25(10):1917-26.
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.
与特发性系统性硬化症（SSc）病理生理学的现有知识相比，本文讨论了有关二氧化硅诱发系统性硬化症（SSc）的临床和实验数据。据报道，约有280例长期接触二氧化硅粉尘后患上SSc的患者，其中一些还伴有矽肺；我们科室对其中111例进行了分析，这是最大的一组病例。基于临床和实验室数据，二氧化硅诱发的SSc和特发性SSc表现出相似的病理生理学特征以及相似的疾病标志物，包括血管受累、免疫异常和细胞外基质代谢失调。实验研究表明，二氧化硅粉尘能够在体外激活微血管内皮细胞、外周血单核细胞和真皮成纤维细胞，其方式与特发性SSc已知的病理生理事件相同。
