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52 基因风险评分在抗纤维化治疗时代识别特发性肺纤维化患者死亡率风险增加的效用。

Utility of the 52-Gene Risk Score to Identify Patients with Idiopathic Pulmonary Fibrosis at Greater Risk of Mortality in the Era of Antifibrotic Therapy.

机构信息

Boehringer Ingelheim Pharma GmbH and Co. KG, Biberach, Germany.

Staburo GmbH, Munich, Germany.

出版信息

Lung. 2024 Oct;202(5):595-599. doi: 10.1007/s00408-024-00742-x. Epub 2024 Sep 6.

DOI:10.1007/s00408-024-00742-x
PMID:39242435
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11427488/
Abstract

PURPOSE

We investigated whether a 52-gene signature was associated with transplant-free survival and other clinically meaningful outcomes in patients with idiopathic pulmonary fibrosis (IPF) in the IPF-PRO Registry, which enrolled patients who were and were not taking antifibrotic therapy.

METHODS

The 52-gene risk signature was implemented to classify patients as being at "high risk" or "low risk" of disease progression and mortality. Transplant-free survival and other outcomes were compared between patients with a low-risk versus high-risk signature.

RESULTS

The 52-gene signature classified 159 patients as at low risk and 86 as at high risk; in these groups, respectively, 56.6% and 51.2% used antifibrotic therapy at enrollment. Among those taking antifibrotic therapy, patients with a low-risk versus high-risk signature were at decreased risk of death, a composite of lung transplant or death, and a composite of decline in DLco % predicted > 15%, lung transplant, or death. Similar results were observed in the overall cohort.

CONCLUSIONS

These data suggest that the 52-gene signature can be used in patients with IPF treated with antifibrotic therapy to distinguish patients at higher risk of disease progression and mortality.

摘要

目的

我们研究了在特发性肺纤维化(IPF)患者的 IPF-PRO 登记处中,52 个基因特征是否与无移植生存和其他有临床意义的结局相关,该登记处纳入了正在接受和未接受抗纤维化治疗的患者。

方法

该 52 个基因风险特征用于将患者分类为疾病进展和死亡率的“高风险”或“低风险”。比较低风险和高风险特征患者之间的无移植生存和其他结局。

结果

该 52 个基因特征将 159 名患者分类为低风险,86 名患者分类为高风险;在这些组中,分别有 56.6%和 51.2%的患者在入组时使用了抗纤维化治疗。在接受抗纤维化治疗的患者中,低风险与高风险特征患者的死亡、肺移植或死亡的复合结局,以及下降>15%、肺移植或死亡的复合结局的风险降低。在整个队列中也观察到了类似的结果。

结论

这些数据表明,52 个基因特征可用于接受抗纤维化治疗的 IPF 患者,以区分疾病进展和死亡率较高的患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5d2/11427488/aff9a2434aa7/408_2024_742_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5d2/11427488/aff9a2434aa7/408_2024_742_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5d2/11427488/aff9a2434aa7/408_2024_742_Fig1_HTML.jpg

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本文引用的文献

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Integrative multi-omics analysis reveals novel idiopathic pulmonary fibrosis endotypes associated with disease progression.整合多组学分析揭示与疾病进展相关的新型特发性肺纤维化表型。
Respir Res. 2023 May 31;24(1):141. doi: 10.1186/s12931-023-02435-0.
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Effect of Antifibrotic Therapy on Survival in Patients With Idiopathic Pulmonary Fibrosis.抗纤维化治疗对特发性肺纤维化患者生存的影响。
Clin Ther. 2023 Apr;45(4):306-315. doi: 10.1016/j.clinthera.2023.03.003. Epub 2023 Mar 28.
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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
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Challenges for Clinical Drug Development in Pulmonary Fibrosis.肺纤维化临床药物研发面临的挑战
Front Pharmacol. 2022 Jan 31;13:823085. doi: 10.3389/fphar.2022.823085. eCollection 2022.
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Blood Transcriptomics Predicts Progression of Pulmonary Fibrosis and Associated Natural Killer Cells.血液转录组学预测肺纤维化的进展及相关自然杀伤细胞。
Am J Respir Crit Care Med. 2021 Jul 15;204(2):197-208. doi: 10.1164/rccm.202008-3093OC.
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Antifibrotic treatment improves clinical outcomes in patients with idiopathic pulmonary fibrosis: a propensity score matching analysis.抗纤维化治疗可改善特发性肺纤维化患者的临床结局:一项倾向评分匹配分析。
Sci Rep. 2020 Sep 24;10(1):15620. doi: 10.1038/s41598-020-72607-1.
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MicroRNA and protein-coding gene expression analysis in idiopathic pulmonary fibrosis yields novel biomarker signatures associated to survival.特发性肺纤维化中 microRNA 和蛋白质编码基因表达分析产生与生存相关的新的生物标志物特征。
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