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[硬皮病的发病机制]

[Pathogenesis of scleroderma].

作者信息

Marie Isabelle

机构信息

Département de médecine interne Hôpital de Boisguillaume 76031 Rouen.

出版信息

Rev Prat. 2002 Nov 1;52(17):1873-81.

Abstract

Systemic sclerosis is a systemic inflammatory disorder, affecting the skin and other organs, especially the lungs and the digestive tract. The pathogenic mechanisms of systemic sclerosis still remain unknown, although there is evidence that many parameters may play a role in the pathogenesis of the disease, including: vascular involvement, immunity impairment, and both environmental and genetic factors. To date, many authors support the hypothesis that, in patients with genetic predisposition, environmental stimuli (occupational factors and/or virus) may lead to vascular dysfunction, particularly, endothelial damage and immune activation, resulting in tissue fibrosis at late stages of the disorder.

摘要

系统性硬化症是一种全身性炎性疾病,会影响皮肤和其他器官,尤其是肺部和消化道。尽管有证据表明许多参数可能在该疾病的发病机制中起作用,包括:血管受累、免疫损伤以及环境和遗传因素,但系统性硬化症的致病机制仍然未知。迄今为止,许多作者支持这样的假说,即对于有遗传易感性的患者,环境刺激(职业因素和/或病毒)可能导致血管功能障碍,特别是内皮损伤和免疫激活,从而在疾病后期导致组织纤维化。

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