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欧洲硬皮病试验与研究数据库中肺功能测试在预测系统性硬化症相关间质性肺病方面的表现

The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis-Interstitial Lung Disease in the European Scleroderma Trial and Research Database.

作者信息

Lepri Gemma, Bruni Cosimo, Tofani Lorenzo, Moggi-Pignone Alberto, Orlandi Martina, Tomassetti Sara, Hughes Michael, Del Galdo Francesco, Irace Rosaria, Distler Oliver, Riccieri Valeria, Allanore Yannick, Gheorghiu Ana Maria, Siegert Elise, De Vries-Bouwstra Jeska, Hachulla Eric, Tikly Mohammed, Damjanov Nemanja, Spertini Francois, Mouthon Luc, Hoffmann-Vold Anna-Maria, Gabrielli Armando, Guiducci Serena, Matucci-Cerinic Marco, Furst Daniel, Bellando-Randone Silvia

机构信息

Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy.

Department of Rheumatology, University Hospital Zurich, University of Zurich, 8006 Zurich, Switzerland.

出版信息

Diagnostics (Basel). 2024 Jan 30;14(3):295. doi: 10.3390/diagnostics14030295.

Abstract

BACKGROUND AND OBJECTIVES

In SSc, ILD is a major cause of morbidity and mortality. We aimed to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) delta change (Δ) and baseline values in predicting the development of SSc-ILD.

METHODS

Longitudinal data of DLCO, FVC, and ILD on the HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t) and after 12 (±4) (t) and 24 (±4) (t) months.

RESULTS

474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t. Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t ( = 0.0031). Neither the mean t to t change (Δ) of DLCO nor the mean t to t FVCΔ predicted the appearance of ILD at t. Investigating the possible role of baseline DLCO and FVC values in predicting ILD appearance after 24 (±4) months, we observed a moderate predictive capability of t DLCO < 80%, stronger than that of FVC < 80%.

CONCLUSIONS

We suggest that an impaired baseline DLCO may be predictive of the appearance of ILD after 2 years of follow-up. This result advances the hypothesis that a reduction in gas exchange may be considered an early sign of lung involvement. However, further rigorous studies are warranted to understand the predictive role of DLCO evaluation in the course of SSc.

摘要

背景与目的

在系统性硬化症(SSc)中,间质性肺疾病(ILD)是发病和死亡的主要原因。我们旨在研究一氧化碳弥散量(DLCO)和用力肺活量(FVC)的变化量(Δ)及基线值在预测SSc-ILD发生方面的表现。

方法

对来自EUSTAR数据库的SSc患者的HRCT上的DLCO、FVC和ILD的纵向数据在基线期(t)以及12(±4)(t)和24(±4)(t)个月后进行评估。

结果

474/17805名患者符合研究条件(403名女性);46名(9.7%)在t期发生ILD。抗拓扑异构酶抗体阳性(117名患者)显示与t期ILD发生有关联(P = 0.0031)。DLCO从t到t的平均变化量(Δ)以及FVC从t到t的平均变化量均未预测到t期ILD的出现。在研究基线DLCO和FVC值在预测24(±4)个月后ILD出现的可能作用时,我们观察到基线DLCO < 80%具有中等预测能力,强于FVC < 80%。

结论

我们认为基线DLCO受损可能预测随访2年后ILD的出现。这一结果推进了如下假设,即气体交换减少可能被视为肺部受累的早期迹象。然而,有必要进行进一步严格研究以了解DLCO评估在SSc病程中的预测作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d92/10855256/56b520fc6fc3/diagnostics-14-00295-g001.jpg

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