Department of Immunology, University of Pittsburgh School of Medicine, 200 Lothrop Street, BST W1052, Pittsburgh, PA 15261, USA.
Immunol Res. 2011 Aug;50(2-3):188-94. doi: 10.1007/s12026-011-8222-1.
Systemic sclerosis (SSc) is a progressive and highly debilitating autoimmune disorder characterized by inflammation, fibrosis, and vascular damage of the connective tissue. T cell-derived cytokines have been implicated in the induction of fibrosis. We found that high levels of the profibrotic type-2 cytokine IL-13 are produced by peripheral blood effector CD8(+) T cells from SSc patients compared to normal controls. This abnormality correlates with increased expression of the transcription factor GATA-3 and the extent of skin fibrosis. Together, the data provide new insights into SSc pathogenesis and identify a specific T cell phenotype that can be used as a biomarker of immune dysfunction in patients with SSc and as a novel therapeutic target for this currently incurable disease.
系统性硬化症（SSc）是一种进行性的、高度衰弱的自身免疫性疾病，其特征为炎症、纤维化和结缔组织的血管损伤。T 细胞衍生的细胞因子已被牵涉到纤维化的诱导中。我们发现，与正常对照相比，来自 SSc 患者的外周血效应 CD8+T 细胞产生高水平的促纤维化 2 型细胞因子 IL-13。这种异常与转录因子 GATA-3 的表达增加和皮肤纤维化的程度相关。这些数据为 SSc 的发病机制提供了新的见解，并确定了一种特定的 T 细胞表型，可作为 SSc 患者免疫功能障碍的生物标志物，并作为目前无法治愈的这种疾病的新的治疗靶点。
