Fibrocytes and the pathogenesis of diffuse parenchymal lung disease.
Fibrocytes and the pathogenesis of diffuse parenchymal lung disease.
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DOI：
10.1186/1755-1536-5-s1-s22
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发表时间：
2012
期刊：
影响因子：
--
通讯作者：
Strieter RM
中科院分区：
文献类型：
--
作者：
Mehrad B;Strieter RM
纤维化是许多慢性肺部疾病发病机制的基础，包括一些肺部感染、气道疾病如支气管扩张和哮喘以及大多数弥漫性实质性肺部疾病。特发性肺纤维化是典型的纤维化肺病，是最常见的弥漫性实质性肺病之一，其特征在于肺功能进行性下降和因呼吸衰竭而过早死亡。由于我们目前无法预测临床恶化和缺乏有效的治疗方法，这种疾病患者的临床管理受到阻碍。纤维细胞是一群骨髓来源的循环祖细胞，其归巢至受损组织并分化成成纤维细胞和肌成纤维细胞，从而促成瘢痕形成。我们总结了支持这些细胞在纤维化肺疾病发病机制中作用的证据。
Fibrosis is fundamental to the pathogenesis of many chronic lung diseases, including some lung infections, airway diseases such as bronchiectasis and asthma, and most of the diffuse parenchymal lung diseases. Idiopathic pulmonary fibrosis, the prototypical fibrotic lung disease, is amongst the most common diffuse parenchymal lung diseases and is characterized by progressive decline in lung function and premature death from respiratory failure. The clinical management of patients with this illness is hampered by our current inability to predict clinical deterioration and lack of an effective therapy. Fibrocytes are a population of bone marrow-derived circulating progenitor cells that home to injured tissues and differentiate into fibroblasts and myofibroblasts, thus contributing to scar formation. We summarize the evidence supporting the role of these cells in the pathogenesis of fibrotic lung diseases.