Derk Chris T, Jimenez Sergio A
Department of Medicine, Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107, USA.
Autoimmun Rev. 2003 Jun;2(4):181-91. doi: 10.1016/s1568-9972(03)00005-3.
Systemic sclerosis (SSc) is an autoimmune disorder of unknown etiology characterized by severe and often progressive cutaneous and visceral fibrosis, pronounced alterations in the microvasculature, and numerous cellular and humoral immune abnormalities. Clinically, SSc is very heterogeneous, encompassing a spectrum ranging from mild limited forms of skin sclerosis with minimal internal organ involvement to severe skin and multiple internal organ fibrosis. Mortality and morbidity in SSc are very high and are directly related to the extent of the fibrotic and microvascular alterations. A better understanding of the pathogenesis of this incurable disorder will help to better target and design effective therapy in the future.
系统性硬化症（SSc）是一种病因不明的自身免疫性疾病，其特征为严重且常呈进行性的皮肤和内脏纤维化、微血管显著改变以及众多细胞和体液免疫异常。临床上，SSc具有高度异质性，涵盖从皮肤硬化程度较轻、内脏器官受累极少的局限性形式到严重皮肤及多内脏器官纤维化的一系列表现。SSc的死亡率和发病率非常高，且与纤维化和微血管改变的程度直接相关。更好地理解这种无法治愈疾病的发病机制将有助于未来更精准地靶向并设计有效的治疗方法。
