Abstract：
The pathogenesis of idiopathic pulmonary fibrosis (IPF) and its histological counterpart, usual interstitial pneumonia (UIP) remains debated. IPF/UIP is a disease characterised by respiratory restriction, and while there have been recent advances in treatment, mortality remains high. Genetic and environmental factors predispose to its development and aberrant alveolar repair is thought to be central. Following alveolar injury, the type II pneumocyte (AEC2) replaces the damaged thin type I pneumocytes. Despite the interstitial fibroblast being considered instrumental in formation of the fibrosis, there has been little consideration for a role for AEC2 in the repair of the septal interstitium. Elastin is a complex protein that conveys flexibility and recoil to the lung. The fibroblast is presumed to produce elastin but there is evidence that the AEC2 may have a role in production or deposition. While the lung is an elastic organ, the role of elastin in repair of lung injury and its possible role in UIP has not been explored in depth. In this paper, pathogenetic mechanisms of UIP involving AEC2 and elastin are reviewed and the possible role of AEC2 in elastin generation is proposed.
摘要：
特发性肺纤维化（IPF）的发病机制及其组织学对应物，通常的间质性肺炎（UIP）仍然存在争议。IPF/UIP是一种以呼吸受限为特征的疾病，虽然最近在治疗方面取得了进展，死亡率仍然很高。遗传和环境因素导致其发育和异常肺泡修复被认为是中心因素。肺泡损伤后，II型肺细胞（AEC2）取代了受损的薄I型肺细胞。尽管间质成纤维细胞被认为是纤维化形成的工具，很少考虑AEC2在间隔间质修复中的作用.弹性蛋白是一种复杂的蛋白质，可将柔韧性和反冲传递到肺部。推测成纤维细胞产生弹性蛋白,但有证据表明AEC2可能在产生或沉积中起作用。虽然肺是一个弹性器官，弹性蛋白在肺损伤修复中的作用及其在UIP中的可能作用尚未得到深入的探讨。在本文中,综述了涉及AEC2和弹性蛋白的UIP的发病机制，并提出了AEC2在弹性蛋白生成中的可能作用。
