Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries.
Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries.
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DOI：
10.1002/art.41669
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发表时间：
2021-05
期刊：
影响因子：
--
通讯作者：
McLaughlin V
中科院分区：
文献类型：
--
作者：
Khanna D;Zhao C;Saggar R;Mathai SC;Chung L;Coghlan JG;Shah M;Hartney J;McLaughlin V
关于结缔组织病（CTD）相关肺动脉高压（PAH）的现代治疗获益程度的数据有限。在这项研究中，我们对随机对照试验（rct）和注册进行了meta分析，以量化这些现代疗法对CTD - PAH患者的益处。在PubMed和Embase数据库中检索2000年1月1日至2019年11月25日期间发表的随机对照试验或注册中心数据的文章。入选标准包括≥30例CTD - PAH患者的多中心研究。对于纳入的RCT，试验必须评估批准的多环芳烃治疗，并且必须报告临床发病率和死亡率或6分钟步行距离的长期风险。要纳入登记，必须报告存活率。随机效应模型用于汇集数据。纳入了11项随机对照试验（共4329例患者，1267例CTD - PAH）和19项注册研究（共9739例患者，4008例CTD - PAH）。与对照组相比，试验性治疗导致总体PAH人群（风险比[HR] 0.64, 95%可信区间[95% CI] 0.54, 0.75; P < 0.001）和CTD - PAH患者（风险比[HR] 0.64, 95% CI 0.51, 0.81; P < 0.001）的临床发病/死亡事件风险降低36%。与所有PAH患者相比，CTD - PAH患者的生存率较低（3年生存率为62%，95% CI为57,67%，95% CI为72%，95% CI为69,75%）。2010年后主要接受治疗的CTD - PAH患者的生存率高于2010年前接受治疗的CTD - PAH患者（3年生存率为73%，95% CI为62,81%，95% CI为65%，95% CI为55,71%）。与总体PAH人群相比，现代治疗在CTD - PAH患者中提供了类似的发病率/死亡率风险降低。总体而言，CTD - PAH患者的死亡风险高于PAH患者，但在过去10年中生存率有所提高，这可能与筛查增加和/或新的治疗方法有关。CTD患者的PAH早期检测和前期强化治疗是有必要的。
Data on the magnitude of benefit of modern therapies for pulmonary arterial hypertension (PAH) in connective tissue disease (CTD)–associated PAH are limited. In this study, we performed meta‐analyses of randomized, controlled trials (RCTs) and registries to quantify the benefit of these modern therapies in patients with CTD‐PAH. The PubMed and Embase databases were searched for articles reporting data from RCTs or registries published between January 1, 2000 and November 25, 2019. Eligibility criteria included multicenter studies with ≥30 CTD‐PAH patients. For an RCT to be included, the trial had to evaluate an approved PAH therapy, and long‐term risks of clinical morbidity and mortality or 6‐minute walk distance had to be reported. For a registry to be included, survival rates had to be reported. Random‐effects models were used to pool the data. Eleven RCTs (total of 4,329 patients; 1,267 with CTD‐PAH) and 19 registries (total of 9,739 patients; 4,008 with CTD‐PAH) were included. Investigational therapy resulted in a 36% reduction in the risk of clinical morbidity/mortality events both in the overall PAH population (hazard ratio [HR] 0.64, 95% confidence interval [95% CI] 0.54, 0.75; P < 0.001) and in CTD‐PAH patients (HR 0.64, 95% CI 0.51, 0.81; P < 0.001) as compared to control subjects. The survival rate was lower in CTD‐PAH patients compared to all PAH patients (survival rate 62%, 95% CI 57, 67% versus 72%, 95% CI 69, 75% at 3 years). The survival rate in CTD‐PAH patients treated primarily after 2010 was higher than that in CTD‐PAH patients treated before 2010 (survival rate 73%, 95% CI 62, 81% versus 65%, 95% CI 59, 71% at 3 years). Modern therapy provides a similar reduction in morbidity/mortality risk in patients with CTD‐PAH when compared to the PAH population overall. Risk of death is higher in CTD‐PAH patients than in those with PAH overall, but survival has improved in the last 10 years, which may be related to increased screening and/or new treatment approaches. Early detection of PAH in patients with CTD and up‐front intensive treatment are warranted.
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影响因子：
158.5
作者：
Galiè, N;Ghofrani, HA;Simonneau, G
通讯作者：
Simonneau, G
影响因子：
24.3
作者：
Hoeper, Marius M.;Kramer, Tilmann;Gruenig, Ekkehard
通讯作者：
Gruenig, Ekkehard
DOI：
10.1016/0197-2456(86)90046-2
发表时间：
1986-09-01
期刊：
CONTROLLED CLINICAL TRIALS
影响因子：
--
作者：
DERSIMONIAN, R;LAIRD, N
通讯作者：
LAIRD, N
影响因子：
37.8
作者：
Galie, Nazzareno;Brundage, Bruce H.;Barst, Robyn J.
通讯作者：
Barst, Robyn J.
影响因子：
158.5
作者：
Galie, N.;Barbera, J. A.;Rubin, L. J.
通讯作者：
Rubin, L. J.