Survival and Prognostic Factors in Systemic Sclerosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis
Survival and Prognostic Factors in Systemic Sclerosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis
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DOI：
10.1002/art.38029
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发表时间：
2013-08-01
影响因子：
--
通讯作者：
Launay, David
中科院分区：
文献类型：
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作者：
Lefevre, Guillaume;Dauchet, Luc;Launay, David
目的肺动脉高压（PH）是系统性硬化症（SSc）的常见并发症。然而，关于生存率及其随时间演变的数据，以及SSc并发PH的预后因素，仍然相互矛盾。本研究的目的是进行系统回顾和荟萃分析的队列研究，以评估合并的生存和预后因素的生存与SSC相关的PH患者。方法对于这个系统回顾和荟萃分析，我们搜索了Medline和EMBase数据库（1960年1月至2012年1月）。所有报告了Ssc相关PH的生存和/或预后因素的队列研究均纳入分析。我们计算了合并的生存率，分析了它们随时间的演变，并确定了生存的预后因素。结果纳入了22项研究，共2，244例SSc相关PH患者。合并的1年、2年和3年生存率为81%。（95%置信区间[95% CI] 79-84%）、64%（95% CI 59-69%）和52%（95% CI 47-58%）。荟萃回归未显示生存率随时间的显著变化，而PH严重程度的基线血流动力学指标与生存率显著相关。在合并肺动脉高压（PAH）的SSc患者中，年龄、男性、一氧化碳弥散量（DLCO）、心包积液和与特发性PAH严重程度相关的经典参数（包括6分钟步行距离、平均肺动脉压、心脏指数和右心房压）是重要的预后因素。DLCO和心包积液是间质性肺疾病相关PH患者的唯一预后因素。结论我们的荟萃分析显示，SSc相关PH患者的汇总3年生存率为52%。PAH严重程度的基线血流动力学指标与SSc相关PAH患者的生存率显著相关，但与患者纳入研究的时间无关。在特发性PAH中通常观察到的所有预后因素，包括6分钟步行距离和右心房压力，也是SSc相关PAH的预后因素。
Objective Pulmonary hypertension (PH) is a frequent and life-limiting complication of systemic sclerosis (SSc). However, data on survival rates and their evolution over time, as well as prognostic factors in SSc complicated by PH, are still conflicting. The aim of this study was to conduct a systematic review and meta-analysis of cohort studies to assess pooled survival and prognostic factors for survival in patients with SSc-associated PH.Methods For this systematic review and meta-analysis, we searched the Medline and EMBase databases (January 1960 to January 2012). All cohort studies in which survival and/or prognostic factors for SSc-associated PH were reported were included in the analysis. We calculated the pooled survival rates and analyzed their evolution over time and identified prognostic factors for survival.Results Twenty-two studies were included, representing a total of 2,244 patients with SSc-associated PH. The pooled 1-, 2-, and 3-year survival rates were 81% (95% confidence interval [95% CI] 79-84%), 64% (95% CI 59-69%), and 52% (95% CI 47-58%), respectively. Meta-regression did not reveal a significant change in survival over time, while baseline hemodynamic measures of PH severity were significantly correlated with survival. In patients with SSc complicated by pulmonary arterial hypertension (PAH), age, male sex, diffusing capacity for carbon monoxide (DLCO), pericardial effusion, and the parameters classically associated with the severity of idiopathic PAH, including the 6-minute walk distance, mean pulmonary artery pressure, cardiac index, and right atrial pressure, were significant prognostic factors. DLCO and pericardial effusion were the only prognostic factors in patients with interstitial lung disease-related PH.Conclusion Our meta-analysis revealed a poor pooled 3-year survival rate of 52% in patients with SSc-associated PH. Baseline hemodynamic measures of PAH severity, but not the period of time during which patients were included in the studies, correlated significantly with survival in patients with SSc-associated PAH. All of the prognostic factors typically observed in idiopathic PAH, including the 6-minute walk distance and right atrial pressure, were also prognostic factors in SSc-associated PAH.