Recent advances in idiopathic pulmonary fibrosis
Recent advances in idiopathic pulmonary fibrosis
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DOI：
10.1378/chest.06-1927
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发表时间：
2007-08-01
期刊：
影响因子：
9.6
通讯作者：
Martinez, Fernando J.
中科院分区：
文献类型：
--
作者：
Noth, Imre;Martinez, Fernando J.
特发性肺纤维化（IPF）仍然是最常见的特发性间质性肺炎，并预示着预后不良。在过去的几年里，在诊断方法和预测结果的能力方面取得了重大进展。高分辨率CT（HRCT）扫描的进步使许多患者无需手术活检即可获得准确诊断。此外，HRCT扫描可以帮助确定预后和确定疾病进展。正确使用HRCT扫描需要多学科的迭代方法，结合所有可用的数据，以达到最终诊断。然而，疾病进展仍存在很大的异质性。肺动脉高压和IPF急性加重对预后有负面影响，并日益成为治疗目标。设计良好的IPF临床试验数量有所增加，这些试验侧重于更具体的靶点。虽然尚未发现治愈方法，但每次试验都扩大了我们对疾病自然过程和靶向治疗影响的理解。在此期间，肺移植似乎可以改善IPF患者亚组的生存率，但仍是唯一的干预措施。本文的目的是回顾对IPF的认识进展以及上述发现的证据。
Idiopathic pulmonary fibrosis (IPF) remains the most common of the idiopathic interstitial pneumonias and portends a poor prognosis. Significant strides have been made in the approach to diagnosis and in the ability to predict outcome in the last few years. Advances in high-resolution CT (HRCT) scanning have allowed an accurate diagnosis obviating the need for surgical biopsy in many patients. Furthermore, HRCT scanning may aid in determining prognosis and identifying disease progression. The appropriate use of the HRCT scan requires a multidisciplinary iterative approach incorporating all available data to reach a final diagnosis. However, there remains great heterogeneity in disease progression. Pulmonary hypertension and acute exacerbations of IPF negatively influence prognosis and are increasingly a target of therapy. There has been an increase in the number of well-designed clinical trials of IPF that have focused on more specific targets. While no cure has yet been found, each trial expands our understanding regarding the natural course of the disease and the impact of targeted therapy. In the interim, lung transplantation, which appears to improve survival in a subset of IPF patients, remains the only intervention. The objective of this article is to review advances in the understanding of IPF and the evidence for the findings outlined above.