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[硬皮病的病理生理学]

[Physiopathology of scleroderma].

作者信息

Fleischmajer R, Perlish J S, Maquart F X, Kalis B

机构信息

Department of Dermatology, Mount Sinai School of Medicine, New York, NY 10029.

出版信息

Ann Dermatol Venereol. 1993;120(2):157-66.

PMID:8363310
Abstract

Scleroderma (systemic sclerosis) is a connective tissue disease characterized by an excessive accumulation of collage in the main body organs. The subsequent progressive fibrosis may result in severe functional impairment of the tissue(s) involved. In this paper, recent advances in the understanding of the disease are reviewed. Particular emphasis is placed on the role played by vascular lesions, inflammatory cell infiltrates, autoimmunity and possibly abnormal secretion of cytokines in the disturbance of connective tissue production. A better knowledge of the pathophysiological process involved in scleroma might lead to the development of new therapeutic approaches.

摘要

硬皮病(系统性硬化症)是一种结缔组织疾病,其特征是在主要身体器官中胶原蛋白过度积聚。随后的进行性纤维化可能导致受累组织出现严重功能障碍。本文综述了对该疾病认识的最新进展。特别强调了血管病变、炎性细胞浸润、自身免疫以及细胞因子可能的异常分泌在结缔组织生成紊乱中所起的作用。对硬皮病所涉及的病理生理过程有更深入的了解可能会带来新治疗方法的发展。

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