Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database.
Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database.
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EUSTAR数据库中系统性硬化症相关间质性肺病患者的进行性间质性肺病
DOI：
10.1136/annrheumdis-2020-217455
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发表时间：
2021-03
影响因子：
27.4
通讯作者：
EUSTAR collaborators
中科院分区：
文献类型：
--
作者：
Hoffmann-Vold AM;Allanore Y;Alves M;Brunborg C;Airó P;Ananieva LP;Czirják L;Guiducci S;Hachulla E;Li M;Mihai C;Riemekasten G;Sfikakis PP;Kowal-Bielecka O;Riccardi A;Distler O;EUSTAR collaborators
使用来自欧洲硬皮病试验和研究（EUSTAR）数据库的长期随访数据，确定系统性硬化症相关间质性肺病（SSc-ILD）患者的总体病程、进展模式和预测进展性间质性肺病（ILD）的风险因素。合格的SSc-ILD患者在EUSTAR数据库中登记，并在基线和12±3个月后测量用力肺活量（FVC）。在具有多个FVC测量值的患者中评估了长期进展性ILD和进展模式。使用多变量混合效应模型分析ILD进展的潜在预测因素。纳入了826例SSc-ILD患者。在12±3个月内，219例（27%）患者出现进展性ILD：中度（FVC下降5%-10%）或显著（FVC下降>10%）。在平均5年随访期间，共有535例（65%）患者有多个可用的FVC测量值。在每个12个月期间，23%至27%的SSc-ILD患者显示出进行性ILD，但只有少数患者在连续期间显示出进展。大多数进展性ILD患者（58%）具有肺功能缓慢下降的模式，稳定/改善的时间段多于下降的时间段，而仅8%的患者表现出快速、持续的FVC下降; 178例（33%）患者未发生FVC下降。5年内FVC下降的最强预测因素是男性、较高的改良Rodnan皮肤评分和反流/吞咽困难症状。SSc-ILD显示出异质性和可变的病程，因此密切监测所有患者非常重要。在FVC下降发生前开始治疗的新治疗概念应旨在预防进展，以避免不可逆的器官损伤。
To identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up. Eligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models. 826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms. SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.
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影响因子：
5.5
作者：
Man, Ada;Davidyock, Todd;Simms, Robert W.
通讯作者：
Simms, Robert W.
DOI：
10.1164/rccm.200808-1359oc
发表时间：
2009-03-01
影响因子：
24.7
作者：
Savarino, Edoardo;Bazzica, Marco;Ghio, Massimo
通讯作者：
Ghio, Massimo
影响因子：
6.9
作者：
Glaspole, Ian N.;Chapman, Sally A.;Corte, Tamera J.
通讯作者：
Corte, Tamera J.
影响因子：
158.5
作者：
Distler, Oliver;Highland, Kristin B.;Maher, Toby M.
通讯作者：
Maher, Toby M.
影响因子：
27.4
作者：
Morgan, C;Knight, C;Silman, AJ
通讯作者：
Silman, AJ