Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment.
Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment.
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DOI：
10.1183/09059180.00008613
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发表时间：
2014-03-01
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在特发性肺纤维化（IPF）中，一些基于大量证据的事实或概念，虽然对于有学问的亚专家来说是隐含的，但以前被忽视和/或没有明确地制定或使更广泛的受众可以访问。IPF与吸烟密切相关，主要是一种衰老性疾病。然而，其原因仍然难以捉摸，因此，它是开发新的有效和安全疗法的最具挑战性的疾病之一。随着吡非尼酮获批用于轻中度IPF患者，IPF的早期诊断是早期治疗的先决条件，并可能改善这种进展性和最终致死性疾病的长期临床结局。作为吸烟者大规模肺癌筛查策略的“副产品”，通过促进间质性肺病的薄层胸部高分辨率计算机断层扫描筛查，可以实现IPF的早期诊断，但过去被忽视的其他技术现在也可用。肺听诊和“维可牢”湿罗音的早期识别被认为是IPF早期诊断的关键组成部分。一项正在进行的研究正在探索使用电子听诊器和高分辨率计算机断层扫描模式获得的听诊肺音之间的相关性。
In idiopathic pulmonary fibrosis (IPF), some facts or concepts based on substantial evidence, whilst implicit for learned subspecialists, have previously been neglected and/or not explicitly formulated or made accessible to a wider audience. IPF is strongly associated with cigarette smoking and is predominantly a disease of ageing. However, its cause(s) remain elusive and, thus, it is one of the most challenging diseases for the development of novel effective and safe therapies. With the approval of pirfenidone for patients with mild-to-moderate IPF, an earlier diagnosis of IPF is a prerequisite for earlier treatment and, potentially, improvement of the long-term clinical outcome of this progressive and ultimately fatal disease. An earlier diagnosis may be achieved in IPF by promoting thin-slice chest high-resolution computed tomography screening of interstitial lung disease as a “by-product” of large-scale lung cancer screening strategies in smokers, but other techniques, which have been neglected in the past, are now available. Lung auscultation and early identification of “velcro” crackles has been proposed as a key component of early diagnosis of IPF. An ongoing study is exploring correlations between lung sounds on auscultation obtained using electronic stethoscopes and high-resolution computed tomography patterns.