Pathogenesis of pulmonary arterial hypertension: lessons from cancer.

Pathogenesis of pulmonary arterial hypertension: lessons from cancer.
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DOI:
10.1183/09059180.00007513
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发表时间:
2013-12
期刊:
European respiratory review : an official journal of the European Respiratory Society
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其他
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尽管导致肺动脉高压(PAH)肺血管床重构的致病机制尚不清楚,但一些与癌变相似的特征已经导致了癌样概念的出现。主要的相似之处在于来自不同组织类型的细胞之间的串扰改变,肺平滑肌和内皮细胞的不明原因的增殖和存活,代谢(糖酵解)的变化,以及与免疫系统的关联。然而,PAH与癌症之间存在着主要的差异,包括没有侵袭和转移,涉及的致病基因以及血管生成障碍和遗传不稳定性的程度。显然,PAH不是一种癌症,但这个类似癌症的概念开辟了一个新的研究领域,并增加了抗增殖和/或肿瘤药物不仅在癌症中发挥治疗作用的可能性,而且在PAH中也发挥了治疗作用。这里讨论了这样的类比和区别。
Although the causal pathomechanisms contributing to remodelling of the pulmonary vascular bed in pulmonary arterial hypertension (PAH) are still unclear, several analogous features with carcinogenesis have led to the emergence of the cancer-like concept. The major similarities concern the altered crosstalk between cells from different tissue types, unexplained proliferation and survival of pulmonary smooth muscle and endothelial cells, the metabolic (glycolytic) shifts, and the association with the immune system. However, major differences between PAH and cancer exist, including the absence of invasion and metastasis, as well as the pathogenic genes involved and the degrees of angiogenesis impairment and genetic instability. It is clear that PAH is not a cancer, but this cancer-like concept has opened a new field of investigation and raises the possibility that antiproliferative and/or oncological drugs may exert therapeutic effects not only in cancer, but also in PAH. Such analogies and differences are discussed here.
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