Gupta P R
Department of Respiratory Medicine, NIMS University, Jaipur, Rajasthan, India.
Lung India. 2014 Jan;31(1):43-6. doi: 10.4103/0970-2113.125899.
Idiopathic pulmonary fibrosis (IPF) is a debilitating lung disease of unknown etiology. Its pathogenesis remains poorly elucidated but aberrant wound healing is central to its pathology. It has a median survival time of 3 to 5 years. None of the treatment modality or drugs tried in its management has so far changed the overall outcome. Recent in vitro and experimental studies have shown that ambroxol hydrochloride exerts several newer actions, namely the surfactant stimulatory, anti-imflammatory and anti-oxidant actions, in addition to its being a secrrtolytic and mucokinetic agent. The anti inflammatory and anti-fibrotic properties of the drug are due to its ability to block the release of oxidant stress markers, cytokines, leukotrienes, MPO activity, hydroxyproline content, nitic oxide and/or collagen I & III mRNA in the local milieu while preserving the SOD and GSH-PX activities. In human studies also, the agent was able to block the expression of TGF-beta and TNF-alpha in plasma and preserving the carbon monoxide diffusion capacity of the lungs in lung cancer patients on radiation therapy. Thus, ambroxol may have the potential to check the dysregulated healing process that is typical of IPF. This, coupled with its safety profile for human use, warrants clinical trials of the drug in the management of IPF.
特发性肺纤维化（IPF）是一种病因不明的使人衰弱的肺部疾病。其发病机制仍未完全阐明，但异常的伤口愈合是其病理的核心。它的中位生存时间为3至5年。迄今为止，在其治疗中尝试的任何治疗方式或药物都没有改变总体预后。最近的体外和实验研究表明，盐酸氨溴索除了具有溶解分泌物和促进黏液运动的作用外，还具有几种新的作用，即表面活性剂刺激作用、抗炎作用和抗氧化作用。该药物的抗炎和抗纤维化特性归因于其能够在局部环境中阻断氧化应激标志物、细胞因子、白三烯、髓过氧化物酶活性、羟脯氨酸含量、一氧化氮和/或I型和III型胶原蛋白mRNA的释放，同时保留超氧化物歧化酶和谷胱甘肽过氧化物酶的活性。在人体研究中，该药物还能够阻断肺癌放疗患者血浆中转化生长因子-β和肿瘤坏死因子-α的表达，并保留肺一氧化碳弥散量。因此，氨溴索可能有潜力抑制IPF典型的愈合过程失调。这一点，再加上其对人体使用的安全性，使得该药物在IPF治疗中的临床试验成为必要。
