Corbel M, Belleguic C, Boichot E, Lagente V
INSERM U456, IFR 97, Université de Rennes I, France.
Cell Biol Toxicol. 2002;18(1):51-61. doi: 10.1023/a:1014471213371.
Pulmonary fibrosis has an aggressive course and is usually fatal an average of 3 to 6 years after the onset of symptoms. Pulmonary fibrosis is associated with deposition of extracellular matrix (ECM) components in the lung interstitium. Matrix metalloproteinases (MMPs) are a major group of proteinases known to regulate the ECM remodeling and so they are hypothesized to be important in the process of lung fibrosis. These led to the concept that modulation of airway remodeling including excessive proteolytic damage of the tissue may be of interest for future treatment. The excessive airway remodeling as a result of an imbalance in the equilibrium of the normal processes of synthesis and degradation of extracellular matrix components could argue in favor of antiprotease treatments. Moreover, these observations emphasize that effective therapies for these disorders must be given early in the natural history of the disease, prior to the development of expensive lung destruction and fibrosis.
肺纤维化病程进展迅速，通常在症状出现后平均3至6年死亡。肺纤维化与肺间质中细胞外基质（ECM）成分的沉积有关。基质金属蛋白酶（MMPs）是一类主要的蛋白酶，已知其可调节ECM重塑，因此推测它们在肺纤维化过程中起重要作用。这些发现引出了一个概念，即调节气道重塑，包括对组织的过度蛋白水解损伤，可能是未来治疗的一个研究方向。由于细胞外基质成分合成与降解的正常过程失衡导致的过度气道重塑，可能支持抗蛋白酶治疗。此外，这些观察结果强调，针对这些疾病的有效治疗必须在疾病自然史的早期给予，即在发生昂贵的肺组织破坏和纤维化之前。
