Pulmonary and Critical Care Medicine, Bruce Webster Professor of Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medical College, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY.
Interstitial Lung Disease Program, Columbia University Medical Center, New York, NY.
Chest. 2018 Oct;154(4):978-979. doi: 10.1016/j.chest.2018.08.1021.
As seen in this CME online activity (available at http://courses.elseviercme.com/694), idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrotic lung disease of unknown cause that is associated with substantial health-care utilization and high rates of mortality. The clinical symptoms of IPF are nonspecific and overlap with many pulmonary and cardiac diseases making differential diagnosis challenging. The American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association (ATS/ERS/JRS/ALAT) guidelines strongly recommend a multidisciplinary approach to the diagnosis of interstitial lung diseases; however, there are several limitations to the feasibility of this approach in clinical practice. Although early diagnosis is relevant to any chronic, progressive, and irreversible disorder, it is crucially important that effective treatments for IPF are prescribed without delay. A window of opportunity may exist during which time treatment can have optimal outcomes. Evidence on the clinical management of IPF is rapidly evolving, and key updates were made to the most recent ATS/ERS/JRS/ALAT guidelines. The widely used combination of prednisone, azathioprine, and N-acetylcysteine has now been associated with increased risk of hospitalization and death compared with placebo in patients with IPF. These treatments and others for IPF have been mostly supportive, but recently pirfenidone and nintedanib have demonstrated efficacy in reducing functional decline and disease progression in IPF. A pooled analysis of three phase 3 studies of pirfenidone found a significant 48% reduction in all-cause mortality, and a pooled analysis of a phase 2 and two phase 3 studies of nintedanib found a significant 43% reduction in on-treatment mortality. As patient exposure to these two new drugs increases, data continue to emerge on how and when to use these medications and on how to manage their side effects. Finally, several medications targeting the fibrotic pathobiology of IPF are currently in development. Given the limited treatment options for IPF, enrollment in a clinical trial may be the best chance to delay or prevent progression of IPF. This CME-certified expert video roundtable from CHEST reviews the ATS/ERS/JRS/ALAT guidelines with a specific focus on accurate and timely diagnosis of IPF and the latest data on the treatment of IPF.
如本 CME 在线活动（可在 http://courses.elseviercme.com/694 上获取）所示，特发性肺纤维化（IPF）是一种原因不明的慢性、进行性、纤维化肺部疾病，其具有大量医疗保健利用和高死亡率。IPF 的临床症状是非特异性的，与许多肺部和心脏疾病重叠，使得鉴别诊断具有挑战性。美国胸科学会/欧洲呼吸学会/日本呼吸学会/拉丁美洲胸科学会（ATS/ERS/JRS/ALAT）指南强烈建议采用多学科方法来诊断间质性肺疾病；然而，在临床实践中，这种方法的可行性存在一些限制。虽然早期诊断与任何慢性、进行性和不可逆的疾病都相关，但重要的是要毫不拖延地开出针对 IPF 的有效治疗方法。在这个时间窗内，治疗可能会产生最佳效果。关于 IPF 临床管理的证据正在迅速发展，并且对最近的 ATS/ERS/JRS/ALAT 指南进行了关键更新。广泛使用的泼尼松、硫唑嘌呤和 N-乙酰半胱氨酸联合治疗与安慰剂相比，现已与 IPF 患者的住院和死亡风险增加相关。这些治疗方法和其他治疗 IPF 的方法大多是支持性的，但最近吡非尼酮和尼达尼布已被证明可减少 IPF 中的功能下降和疾病进展。吡非尼酮的三项 3 期研究的汇总分析发现全因死亡率降低了 48%，尼达尼布的一项 2 期和两项 3 期研究的汇总分析发现治疗期间死亡率降低了 43%。随着患者对这两种新药的接触增加，有关如何以及何时使用这些药物以及如何管理其副作用的持续出现数据。最后，目前有几种针对 IPF 纤维化病理生物学的药物正在开发中。鉴于 IPF 的治疗选择有限，参加临床试验可能是延迟或预防 IPF 进展的最佳机会。CHEST 提供的本 CME 认证的专家视频小组讨论重点介绍了 ATS/ERS/JRS/ALAT 指南，特别关注 IPF 的准确和及时诊断以及 IPF 治疗的最新数据。
