肺动脉高压（Pulmonary arterial hypertension, PAH）是结缔组织病的严重并发症，血管过度增殖和炎症是主要发病机制。高半胱氨酸蛋白61（Cysteine rich 61 ,Cyr61) 是重要的血管刺激因子，可促进炎症反应、细胞增殖，并在多种疾病的发病中起关键作用。我们的前期研究发现系统性红斑狼疮及系统性硬化相关PAH患者血浆及野百合碱诱导的PAH大鼠模型肺动脉壁Cyr61表达均增加，提示Cyr61可能在参与结缔组织病相关PAH的发病。我们推测，Cyr61通过促进血管增殖和肺组织炎症参与了结缔组织病相关PAH的发病。因此，我们将针对Cyr61对野百合碱诱导的PAH大鼠肺血管平滑肌细胞增殖以及炎症过程的影响进行研究，并探讨其作用机制。目前此研究在国内外尚属首次，希望能够通过此研究进一步加深对于结缔组织病相关PAH发病机制的理解,为寻找新的治疗靶点提供重要线索。

Pulmonary arterial hypertension（PAH）is a severe complication of connective tissue disease. Proliferation and inflammation have been confirmed to be involved in the pathogenesis of connective tissue disease associated PAH. Cysteine-rich 61（Cyr61）is important in promoting angiogenesis, cell proliferation, and inflammatory processes. It has been proved that Cyr61 plays a povital role in varieties of biological processes. Our preliminary study demonstrated that expression of Cyr61 was significantly elevated in the plasma of systemic lupus erythematosus and systemic sclerosis associated PAH and in the wall of pulmonary artery in monocrotaline rat models of PAH, indicating Cyr61 may play an important role in the pathogenesis of connective tissue disease associated PAH, therefore, we hypothesize that Cyr61 is involved in the pathogenesis of connective tissue disease associated PAH by stimulating vascular proliferation and promoting inflammation in lung. To test this hypotheses, we will set out to look at the impact of Cyr61 on the cell proliferation and inflammation of pulmonary vessels, and try to further investigate the possible mechanism in this study. Our study will help to further understand the pathogenesis of CTD-PAH, and also may provide important clues to finding potential therapeutic targets in the future. The study on Cyr61 in the pathogenesis of connective tissue disease asssociated PAH has never been reported worldwide.