Institut Cochin, INSERM U1016, Paris, France.
Clin Rev Allergy Immunol. 2013 Feb;44(1):31-8. doi: 10.1007/s12016-011-8265-z.
Pulmonary arterial hypertension is characterized by a remodeling of pulmonary arteries with endothelial cell, fibroblast, and vascular smooth muscle cell activation and proliferation. Since pulmonary arterial hypertension occurs frequently in autoimmune conditions such as systemic sclerosis, inflammation and autoimmunity have been suspected to play a critical role in both idiopathic pulmonary arterial hypertension and systemic sclerosis-associated pulmonary arterial hypertension. High levels of pro-inflammatory cytokines such as interleukin-1 and interleukin-6, platelet-derived growth factor, or macrophage inflammatory protein 1 have been found in lung samples of patients with pulmonary arterial hypertension, along with inflammatory cell infiltrates mainly composed of macrophages and dendritic cells, T and B lymphocytes. In addition, circulating autoantibodies are found in the peripheral blood of patients. Thus, autoimmunity and inflammation probably play a role in the development of pulmonary arterial hypertension. In this setting, it would be important to set-up new experimental models of pulmonary arterial hypertension, in order to define novel therapeutics that specifically target immune disturbances in this devastating condition.
肺动脉高压的特征是肺血管出现重塑，伴有内皮细胞、成纤维细胞和血管平滑肌细胞的激活和增殖。由于肺动脉高压常发生于系统性硬化等自身免疫性疾病中，因此炎症和自身免疫被怀疑在特发性肺动脉高压和系统性硬化相关肺动脉高压中都发挥了关键作用。在肺动脉高压患者的肺组织样本中发现了高水平的促炎细胞因子，如白细胞介素-1 和白细胞介素-6、血小板衍生生长因子或巨噬细胞炎症蛋白 1，同时还存在主要由巨噬细胞和树突状细胞、T 和 B 淋巴细胞组成的炎症细胞浸润。此外，还在患者的外周血中发现了循环自身抗体。因此，自身免疫和炎症可能在肺动脉高压的发生发展中起作用。在这种情况下，建立新的肺动脉高压实验模型很重要，以便确定针对这种破坏性疾病中免疫紊乱的新疗法。
