Yazawa Norihito, Fujimoto Manabu, Tamaki Kunihiko
Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
Clin Rev Allergy Immunol. 2007 Oct;33(1-2):107-12. doi: 10.1007/s12016-007-8009-2.
Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by extensive fibrotic changes in various organs, including skin and lung. Although the etiology of SSc remains unknown, three major abnormalities, abnormal humoral immunity, microvasculature, and fibroblast dysfunctions are considered to play important roles. Significant progress has been made in understanding the pathogenesis on SSc, and has been also providing clues to the treatment for this disease. This review summarizes recent advances on the pathogenesis and new therapeutic strategy for SSc.
系统性硬化症(SSc)是一种全身性自身免疫性疾病,其特征是包括皮肤和肺在内的各种器官发生广泛的纤维化改变。尽管SSc的病因尚不清楚,但三种主要异常,即异常的体液免疫、微血管病变和成纤维细胞功能障碍被认为起着重要作用。在理解SSc的发病机制方面已经取得了重大进展,也为该疾病的治疗提供了线索。本综述总结了SSc发病机制和新治疗策略的最新进展。