Kayser Cristiane, Fritzler Marvin J
Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo , São Paulo , Brazil.
Faculty of Medicine, Cumming School of Medicine, University of Calgary , Calgary, AB , Canada.
Front Immunol. 2015 Apr 15;6:167. doi: 10.3389/fimmu.2015.00167. eCollection 2015.
Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, and cutaneous and visceral fibrosis. Serum autoantibodies directed to multiple intracellular antigens are present in more than 95% of patients and are considered a hallmark of SSc. They are helpful biomarkers for the early diagnosis of SSc and are associated with distinctive clinical manifestations. With the advent of more sensitive, multiplexed immunoassays, new and old questions about the relevance of autoantibodies in SSc are emerging. In this review, we discuss the clinical relevance of autoantibodies in SSc emphasizing the more recently published data. Moreover, we will summarize recent advances regarding the stability of SSc autoantibodies over the course of disease, whether they are mutually exclusive and their potential roles in the disease pathogenesis.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为血管异常以及皮肤和内脏纤维化。超过95%的患者体内存在针对多种细胞内抗原的血清自身抗体，这些抗体被视为SSc的标志。它们是SSc早期诊断的有用生物标志物，并与独特的临床表现相关。随着更灵敏的多重免疫测定法的出现，关于自身抗体在SSc中的相关性的新老问题不断涌现。在本综述中，我们讨论了SSc中自身抗体的临床相关性，并着重强调了最近发表的数据。此外，我们将总结关于SSc自身抗体在疾病过程中的稳定性、它们是否相互排斥以及它们在疾病发病机制中的潜在作用的最新进展。
