Inflammation in Pulmonary Arterial Hypertension
Inflammation in Pulmonary Arterial Hypertension
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DOI：
10.1378/chest.11-0793
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发表时间：
2012-01-01
期刊：
影响因子：
9.6
通讯作者：
Humbert, Marc
中科院分区：
文献类型：
--
作者：
Price, Laura C.;Wort, S. John;Humbert, Marc
肺动脉高压（Pulmonary arterial hypertension， PAH）的特点是毛细血管前肺动脉的肺血管重构，血管细胞过度增生。虽然确切的病理生理机制尚不清楚，但越来越多的证据表明炎症起着重要作用。首先，PAH患者的病理标本显示血管周围炎症细胞积聚，包括巨噬细胞、树突状细胞、T淋巴细胞和B淋巴细胞以及肥大细胞。其次，某些细胞因子和趋化因子的循环水平升高，这可能与较差的临床结果相关。第三，某些炎症条件，如结缔组织疾病与多环芳烃发病率增加有关。最后，治疗潜在的炎症可以减轻相关的多环芳烃。潜在的病理机制可能是“多重打击”和复杂的。例如，炎症反应可能受骨形态发生蛋白受体2 （BMPR II）状态的调节，反过来，BMPR II的表达可以被某些细胞因子改变。虽然抗炎治疗在某些结缔组织疾病相关的PAH中有效，但这种方法在特发性PAH （iPAH）中尚未经过测试。抗炎治疗在iPAH中的潜在益处是重要的，需要进一步研究。胸部2012;141 (1): 210 - 221
Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling of the precapillary pulmonary arteries, with excessive proliferation of vascular cells. Although the exact pathophysiology remains unknown, there is increasing evidence to suggest an important role for inflammation. Firstly, pathologic specimens from patients with PAH reveal an accumulation of perivascular inflammatory cells, including macrophages, dendritic cells, T and B lymphocytes, and mast cells. Secondly, circulating levels of certain cytokines and chemokines are elevated, and these may correlate with a worse clinical outcome. Thirdly, certain inflammatory conditions such as connective tissue diseases are associated with an increased incidence of PAH. Finally, treatment of the underlying inflammatory condition may alleviate the associated PAH. Underlying pathologic mechanisms are likely to be "multihit" and complex. For instance, the inflammatory response may be regulated by bone morphogenetic protein receptor type 2 (BMPR II) status, and, in turn, BMPR II expression can be altered by certain cytokines. Although antiinflammatory therapies have been effective in certain connective-tissue-disease-associated PAH, this approach is untested in idiopathic PAH (iPAH). The potential benefit of antiinflammatory therapies in iPAH is of importance and requires further study. CHEST 2012; 141(1):210-221