Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives
Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives
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DOI：
10.1183/13993003.01887-2018
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发表时间：
2019-01-01
影响因子：
24.3
通讯作者：
Rabinovitch, Marlene
中科院分区：
文献类型：
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作者：
Humbert, Marc;Guignabert, Christophe;Rabinovitch, Marlene
临床和转化研究在促进我们对肺动脉高压（PH）的理解方面发挥了重要作用，包括肺动脉高压和其他形式的PH与严重的血管重塑（例如，G.慢性血栓栓塞性PH和肺静脉闭塞性疾病）。然而，PH仍然是一种无法治愈的疾病，死亡率很高，强调需要更好地将新的科学知识转移到医疗保健干预措施中。在此，我们回顾了病理学的最新研究结果（质疑各种形式的PH到前或后毛细血管参与肺血管的严格形态分类）和细胞机制，有助于与各种形式的PH相关的肺血管重塑的发生和进展。我们还讨论了如何改善管理，支持和优化药物开发在这一研究领域。
Clinical and translational research has played a major role in advancing our understanding of pulmonary hypertension (PH), including pulmonary arterial hypertension and other forms of PH with severe vascular remodelling (e. g. chronic thromboembolic PH and pulmonary veno- occlusive disease). However, PH remains an incurable condition with a high mortality rate, underscoring the need for a better transfer of novel scientific knowledge into healthcare interventions. Herein, we review recent findings in pathology (with the questioning of the strict morphological categorisation of various forms of PH into pre- or post- capillary involvement of pulmonary vessels) and cellular mechanisms contributing to the onset and progression of pulmonary vascular remodelling associated with various forms of PH. We also discuss ways to improve management and to support and optimise drug development in this research field.