特发性肺纤维化
肺
肺纤维化
医学
发病机制
细胞外基质
纤维化
间质性肺病
肺炎
病理
免疫学
生物
内科学
细胞生物学
作者
Isis E. Fernandez,Oliver Eickelberg
出处
期刊:The Lancet
[Elsevier BV]
日期:2012-08-01
卷期号:380 (9842): 680-688
被引量:394
标识
DOI:10.1016/s0140-6736(12)61144-1
摘要
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.
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