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特发性肺纤维化的研究进展及治疗。

Progress in Understanding and Treating Idiopathic Pulmonary Fibrosis.

机构信息

Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA; email:

Department of Veterans Affairs Medical Center, Nashville, Tennessee 37212, USA.

出版信息

Annu Rev Med. 2019 Jan 27;70:211-224. doi: 10.1146/annurev-med-041317-102715.

Abstract

This is a time of substantial progress in the evaluation and care of patients with idiopathic pulmonary fibrosis (IPF). In addition to the approval and widespread availability of the first IPF-specific therapies, there have been improvements in imaging interpretation and lung biopsy methods to enable more expeditious and more accurate diagnosis. Recent advances in identifying genetic factors that underlie susceptibility to IPF and affect prognosis have raised the possibility of personalized therapeutic approaches in the future. Further, evolving work is elucidating novel mechanisms influencing epithelial, mesenchymal, and inflammatory cell responses during the injury-repair process, thus advancing understanding of disease pathogenesis. As analytic approaches mature, the field is now poised to harness the power of rapidly advancing "omics" technologies to further accelerate progress.

摘要

这是评估和治疗特发性肺纤维化(IPF)患者方面取得重大进展的时期。除了首个 IPF 特异性治疗药物的批准和广泛应用外,在成像解读和肺活检方法方面也取得了进步,从而能够更快速、更准确地诊断。在确定导致 IPF 易感性和影响预后的遗传因素方面的最新进展,使未来有可能采用个性化的治疗方法。此外,正在进行的研究阐明了在损伤修复过程中影响上皮细胞、间充质细胞和炎症细胞反应的新机制,从而深入了解疾病的发病机制。随着分析方法的成熟,该领域现在准备利用快速发展的“组学”技术的力量来进一步加速进展。

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