Swigris Jeffrey J, Brown Kevin K
Interstitial Lung Disease Program, National Jewish Medical and Research Center, Denver, Colorado 80206, USA.
J Bras Pneumol. 2006 May-Jun;32(3):249-60.
Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These breakthroughs, combined with the increased general availability of therapeutic trials, hold the promise of a brighter future for idiopathic pulmonary fibrosis patients. For example, we now have a more comprehensive understanding of the diagnostic criteria and natural history of the disease. Several studies have shown that simple measurement of pulmonary physiology or gas exchange can be used to predict patient survival. By identifying several molecular pathways that play significant roles in the pathogenesis of idiopathic pulmonary fibrosis, investigators have produced a growing list of novel potential therapeutic targets for the disease. Several prospective, controlled therapeutic trials have been conducted. Others are ongoing or are still in the planning stages. These efforts have advanced our current knowledge of idiopathic pulmonary fibrosis and have raised new important questions, as well as having generated the interest and momentum needed to gain additional ground in the fight against this challenging disease. This article offers the reader a view of the recent advances in idiopathic pulmonary fibrosis research, with a focus on natural history, pathogenesis and treatment.
尽管特发性肺纤维化仍然是一个毁灭性的诊断，但最近的进展增进了我们对这种疾病诸多方面的理解。这些突破，再加上治疗试验总体上更易获得，为特发性肺纤维化患者带来了更光明未来的希望。例如，我们现在对该疾病的诊断标准和自然史有了更全面的认识。多项研究表明，简单的肺生理学或气体交换测量可用于预测患者的生存情况。通过确定在特发性肺纤维化发病机制中起重要作用的几种分子途径，研究人员列出了越来越多针对该疾病的新型潜在治疗靶点。已经进行了多项前瞻性对照治疗试验。其他试验正在进行中或仍处于规划阶段。这些努力推进了我们目前对特发性肺纤维化的认识，提出了新的重要问题，也产生了在对抗这种具有挑战性的疾病方面取得更多进展所需的兴趣和动力。本文向读者展示了特发性肺纤维化研究的最新进展，重点是自然史、发病机制和治疗。
