New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis
New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis
复制标题
DOI：
10.1016/s0140-6736(12)61144-1
复制
发表时间：
2012-08-18
期刊：
影响因子：
168.9
通讯作者：
Eickelberg, Oliver
中科院分区：
文献类型：
--
作者：
Fernandez, Isis E.;Eickelberg, Oliver
特发性肺纤维化是一种严重的进行性慢性肺部疾病，其特征是外周肺细胞组成改变和内平衡，导致细胞外基质过度积累，最终导致肺功能丧失。它是预后最差的间质性肺炎，诊断后3-5年死亡率为50%。在过去的十年中，研究人员已经描述了一些与特发性肺纤维化发病机制有关的新的细胞和分子机制以及信号通路，从而确定了新的治疗靶点。这些进步有望在未来提高这种疾病患者的存活率和改善生活质量。
Idiopathic pulmonary fibrosis is a serious and progressive chronic lung disease that is characterised by altered cellular composition and homoeostasis in the peripheral lung, leading to excessive accumulation of extracellular matrix and, ultimately, loss of lung function. It is the interstitial pneumonia with the worst prognosis-mortality 3-5 years after diagnosis is 50%. During the past decade, researchers have described several novel cellular and molecular mechanisms and signalling pathways implicated in the pathogenesis of idiopathic pulmonary fibrosis, resulting in the identification of new therapeutic targets. These advances will hopefully result in increased survival rates and improved quality of life for patients with this disorder in future.