Screening for Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis Clinical Characteristics at Diagnosis and Long-Term Survival
Screening for Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis Clinical Characteristics at Diagnosis and Long-Term Survival
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DOI：
10.1002/art.30541
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发表时间：
2011-11-01
影响因子：
--
通讯作者：
Hachulla, Eric
中科院分区：
文献类型：
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作者：
Humbert, Marc;Yaici, Azzedine;Hachulla, Eric
客观的。肺动脉高压（PAH）是系统性硬化症（SSc）的一种严重的、限制生命的并发症。指南建议早期检测和管理 SSc-PAH。然而，人们对检测项目对 SSc-PAH 患者的影响知之甚少。本研究旨在评估 SSc-PAH 患者诊断时的临床特征及其长期结果。方法。对来自同一管理时代（2002/2003）的两个 SSc-PAH 患者事件队列进行了研究。第一个队列（指定为常规实践队列）包括有症状 SSc 的连续成年患者，这些患者在被招募到法国 PAH 登记处时通过右侧心导管插入术 (RHC) 诊断为 PAH。第二组（指定为检测组）由连续的 SSc 患者组成，他们进入系统性 PAH 检测计划，随后在 RHC 上发现患有 PAH。对各队列之间诊断 PAH 时的临床特征和随后的 8 年死亡率进行比较。结果。每个队列有 16 名患者。在 PAH 诊断时，与常规实践队列中的患者相比，检测队列中的患者患有较少的晚期肺血管疾病，这可以从更多的患者处于纽约心脏协会 I 级和 II 级、较低的平均肺动脉压和肺血管阻力指数以及较高的心输出量中得到证明。检测队列中的患者接受利尿剂和华法林的可能性较小，但各队列之间接受 PAH 特异性治疗的暴露情况没有差异。常规练习队列中的 1 年、3 年、5 年和 8 年生存率分别为 75%、31%、25% 和 17%，而检测队列中分别为 100%、81%、73% 和 64% (P = 0.0037)。结论。与常规临床实践中的患者相比，SSc 中的 PAH 检测项目能够识别病情较轻的患者，从而实现早期治疗。
Objective. Pulmonary arterial hypertension (PAH) is a severe, life-limiting complication of systemic sclerosis (SSc). Guidelines recommend early detection and management of SSc-PAH. However, little is known about the impact of detection programs on patients with SSc-PAH. This study was undertaken to assess the clinical characteristics of patients with SSc-PAH at diagnosis and their long-term outcomes.Methods. Two incident cohorts of patients with SSc-PAH from the same management era (2002/2003) were studied. The first cohort (designated the routine practice cohort) included consecutive adult patients with symptomatic SSc in whom a diagnosis of PAH was made by right-sided heart catheterization (RHC) at the time of recruitment into the French PAH Registry. The second cohort (designated the detection cohort) comprised consecutive patients with SSc who entered a systematic PAH detection program and were subsequently found to have PAH on RHC. Clinical characteristics at diagnosis of PAH and subsequent 8-year mortality were compared between the cohorts.Results. There were 16 patients in each cohort. At the time of PAH diagnosis, patients in the detection cohort had less advanced pulmonary vascular disease compared with patients in the routine practice cohort, as evidenced by more patients being in New York Heart Association class I and class II, a lower mean pulmonary artery pressure and pulmonary vascular resistance index, and a higher cardiac output. Patients in the detection cohort were less likely to receive diuretics and warfarin, but there was no difference in exposure to PAH-specific therapies between the cohorts. The 1-, 3-, 5-, and 8-year survival rates were 75%, 31%, 25%, and 17%, respectively, in the routine practice cohort compared with 100%, 81%, 73%, and 64%, respectively, in the detection cohort (P = 0.0037).Conclusion. Compared with patients in routine clinical practice, PAH detection programs in SSc are able to identify patients with milder forms of the disease, allowing earlier management.