Alveolar epithelial disintegrity in pulmonary fibrosis
Alveolar epithelial disintegrity in pulmonary fibrosis
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DOI：
10.1152/ajplung.00115.2016
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发表时间：
2016-08-01
影响因子：
4.9
通讯作者：
Thannickal, Victor J.
中科院分区：
文献类型：
--
作者：
Kulkarni, Tejaswini;de Andrade, Joao;Thannickal, Victor J.
特发性肺纤维化（IPF）是一种慢性肺部疾病，其特征是肺功能进行性下降，导致显著的发病率和死亡率。目前IPF发病机制的概念主要集中在慢性暴露于香烟烟雾或环境毒素后上皮细胞修复失调和上皮-间充质通讯改变以及细胞外基质沉积。近年来，越来越多的注意力已经指向细胞间连接复合物在决定肺部疾病中上皮细胞的特异性性质中的作用。此外，最近的全基因组关联研究表明，预测上皮细胞功能障碍的特定遗传变异可能赋予散发性特发性肺纤维化发展的易感性。许多与肺纤维化和家族性间质性肺炎相关的遗传性疾病与上皮完整性的丧失有关。然而，与上皮细胞缺陷相关的肺外临床综合征与肺纤维化发展之间的潜在联系尚不清楚。在此，我们报告一个遗传性黏膜上皮异常增生的病例，在高解析度电脑断层扫描上表现为肺纤维化和肺气肿。这个病例说明了一个更普遍的概念，上皮不完整的发展纤维化肺疾病，这是更详细的探讨，在这篇评论文章。
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by progressive decline in lung function, resulting in significant morbidity and mortality. Current concepts of the pathogenesis of IPF primarily center on dysregulated epithelial cell repair and altered epithelial-mesenchymal communication and extracellular matrix deposition following chronic exposure to cigarette smoke or environmental toxins. In recent years, increasing attention has been directed toward the role of the intercellular junctional complex in determining the specific properties of epithelia in pulmonary diseases. Additionally, recent genomewide association studies suggest that specific genetic variants predictive of epithelial cell dysfunction may confer susceptibility to the development of sporadic idiopathic pulmonary fibrosis. A number of genetic disorders linked to pulmonary fibrosis and familial interstitial pneumonias are associated with loss of epithelial integrity. However, the potential links between extrapulmonary clinical syndromes associated with defects in epithelial cells and the development of pulmonary fibrosis are not well understood. Here, we report a case of hereditary mucoepithelial dysplasia that presented with pulmonary fibrosis and emphysema on high-resolution computed tomography. This case illustrates a more generalizable concept of epithelial disintegrity in the development of fibrotic lung diseases, which is explored in greater detail in this review article.