The Processes and Mechanisms of Cardiac and Pulmonary Fibrosis.

The Processes and Mechanisms of Cardiac and Pulmonary Fibrosis.
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DOI:
10.3389/fphys.2017.00777
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发表时间:
2017
影响因子:
4
通讯作者:
Boyle AJ
Boyle AJ
中科院分区:
医学2区
文献类型:
--
作者:
Murtha LA;Schuliga MJ;Mabotuwana NS;Hardy SA;Waters DW;Burgess JK;Knight DA;Boyle AJ

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纤维化是纤维结缔组织对损伤的反应。它的特点是细胞外基质成分,特别是胶原蛋白,在损伤部位的积累。纤维化是一种适应性反应,是伤口愈合和组织修复的重要组成部分。然而,它的持续激活是非常有害的,是包括心血管和呼吸系统疾病在内的许多疾病状态的共同最终途径。在世界范围内,纤维化疾病每年造成80多万人死亡,占总死亡人数的45%左右。随着人口的老龄化,纤维化疾病的发病率和随后与纤维化相关的死亡人数将进一步上升。尽管纤维化是一系列疾病状态下发病率和死亡率的公认原因,但目前还没有可行的治疗方法来逆转慢性纤维化的影响。许多易感因素有助于纤维化的发展。特别是生物老化,干扰了受损组织的修复,加速了向病理性重塑的过渡,而不是一个分解和再生的过程。当纤维化以不受控制的方式发展时,它会导致受影响组织的不可逆硬化,这可能导致器官功能障碍和死亡。进一步研究纤维化的机制对于阐明新的、急需的治疗靶点是必要的。心脏和肺纤维化在纤维化相关死亡中占很大比例。长期以来,人们已经确定,当涉及到健康和疾病时,心脏和肺在功能和地理上是联系在一起的,因此,探索导致每个器官纤维化的过程和机制,这一综述的重点,可能有助于突出潜在的治疗研究途径。
Fibrosis is the formation of fibrous connective tissue in response to injury. It is characterized by the accumulation of extracellular matrix components, particularly collagen, at the site of injury. Fibrosis is an adaptive response that is a vital component of wound healing and tissue repair. However, its continued activation is highly detrimental and a common final pathway of numerous disease states including cardiovascular and respiratory disease. Worldwide, fibrotic diseases cause over 800,000 deaths per year, accounting for ~45% of total deaths. With an aging population, the incidence of fibrotic disease and subsequently the number of fibrosis-related deaths will rise further. Although, fibrosis is a well-recognized cause of morbidity and mortality in a range of disease states, there are currently no viable therapies to reverse the effects of chronic fibrosis. Numerous predisposing factors contribute to the development of fibrosis. Biological aging in particular, interferes with repair of damaged tissue, accelerating the transition to pathological remodeling, rather than a process of resolution and regeneration. When fibrosis progresses in an uncontrolled manner, it results in the irreversible stiffening of the affected tissue, which can lead to organ malfunction and death. Further investigation into the mechanisms of fibrosis is necessary to elucidate novel, much needed, therapeutic targets. Fibrosis of the heart and lung make up a significant proportion of fibrosis-related deaths. It has long been established that the heart and lung are functionally and geographically linked when it comes to health and disease, and thus exploring the processes and mechanisms that contribute to fibrosis of each organ, the focus of this review, may help to highlight potential avenues of therapeutic investigation.
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