Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis
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DOI：
10.1016/s0140-6736(11)60052-4
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发表时间：
2011-12-03
期刊：
影响因子：
168.9
通讯作者：
Selman, Moises
中科院分区：
文献类型：
--
作者：
King, Talmadge E., Jr.;Pardo, Annie;Selman, Moises
特发性肺纤维化是一种毁灭性的，与年龄相关的肺部疾病，原因不明，治疗选择很少。这种疾病曾被认为是一种慢性炎症过程，但目前的证据表明，纤维化反应是由异常激活的肺泡上皮细胞（AEC）驱动的。这些细胞产生介体，介体通过驻留间充质细胞的增殖、循环纤维细胞的吸引和上皮向间充质转化的刺激诱导成纤维细胞和肌成纤维细胞灶的形成。成纤维细胞和肌成纤维细胞病灶分泌过量的细胞外基质，主要是胶原，导致肺结构的瘢痕形成和破坏。特发性肺纤维化与衰老和异常上皮活化的联系机制尚不清楚;有证据表明，发育途径的异常重演和表观遗传变化起作用。在这次研讨会上，我们回顾了特发性肺纤维化发病机制中涉及的临床过程、治疗选择和潜在机制的最新数据。
Idiopathic pulmonary fibrosis is a devastating, age-related lung disease of unknown cause that has few treatment options. This disease was once thought to be a chronic inflammatory process, but current evidence indicates that the fibrotic response is driven by abnormally activated alveolar epithelial cells (AECs). These cells produce mediators that induce the formation of fibroblast and myofibroblast foci through the proliferation of resident mesenchymal cells, attraction of circulating fibrocytes, and stimulation of the epithelial to mesenchymal transition. The fibroblast and myofibroblast foci secrete excessive amounts of extracellular matrix, mainly collagens, resulting in scarring and destruction of the lung architecture. The mechanisms that link idiopathic pulmonary fibrosis with ageing and aberrant epithelial activation are unknown; evidence suggests that the abnormal recapitulation of developmental pathways and epigenetic changes have a role. In this Seminar, we review recent data on the clinical course, therapeutic options, and underlying mechanisms thought to be involved in the pathogenesis of idiopathic pulmonary fibrosis.