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间质性肺疾病诊断与管理的最新进展

Update in diagnosis and management of interstitial lung disease.

作者信息

Mikolasch Theresia A, Garthwaite Helen S, Porter Joanna C

机构信息

UCL Respiratory, Univeristy College London and Interstitial Lung Disease Service, University College London NHS Foundation Trust, London, UK.

UCL Respiratory, University College London and Interstitial Lung Disease Service, University College London NHS Foundation Trust, London, UK

出版信息

Clin Med (Lond). 2016 Dec;16(Suppl 6):s71-s78. doi: 10.7861/clinmedicine.16-6-s71.

Abstract

The field of interstitial lung disease (ILD) has undergone significant evolution in recent years, with an increasing incidence and more complex, ever expanding disease classification. In their most severe forms, these diseases lead to progressive loss of lung function, respiratory failure and eventually death. Despite notable advances, progress has been challenged by a poor understanding of pathological mechanisms and patient heterogeneity, including variable progression. The diagnostic pathway is thus being continually refined, with the introduction of tools such as transbronchial cryo lung biopsy and a move towards genetically aided, precision medicine. In this review, we focus on how to approach a patient with ILD and the diagnostic process.

摘要

近年来,间质性肺疾病(ILD)领域发生了重大变革,发病率不断上升,疾病分类愈发复杂且持续扩展。这些疾病最严重的形式会导致肺功能逐渐丧失、呼吸衰竭,最终致人死亡。尽管取得了显著进展,但对病理机制的理解不足以及患者的异质性,包括病情进展的差异,给研究带来了挑战。因此,诊断途径在不断完善,引入了经支气管冷冻肺活检等工具,并朝着基因辅助的精准医学方向发展。在本综述中,我们重点关注如何诊治ILD患者以及诊断过程。

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