系统性硬化症患者肺动脉高压的筛查：诊断时的临床特征及长期生存率

Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival.

作者信息

Humbert Marc, Yaici Azzedine, de Groote Pascal, Montani David, Sitbon Olivier, Launay David, Gressin Virginie, Guillevin Loïc, Clerson Pierre, Simonneau Gérald, Hachulla Eric

机构信息

Université Paris-Sud, Le Kremlin-Bicêtre, France; AP-HP Hôpital Antoine Béclère, Clamart, France.

出版信息

Arthritis Rheum. 2011 Nov;63(11):3522-30. doi: 10.1002/art.30541.

DOI:10.1002/art.30541

PMID:21769843

Abstract

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.

摘要

目的

肺动脉高压（PAH）是系统性硬化症（SSc）的一种严重的、危及生命的并发症。指南建议对SSc-PAH进行早期检测和管理。然而，关于检测项目对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预后。

方法

对来自同一管理时代（2002/2003年）的两个SSc-PAH患者发病队列进行研究。第一个队列（指定为常规实践队列）包括连续的有症状的SSc成年患者，这些患者在被纳入法国PAH注册中心时通过右心导管检查（RHC）确诊为PAH。第二个队列（指定为检测队列）包括连续的SSc患者，他们进入了一个系统性PAH检测项目，随后通过RHC被发现患有PAH。比较两个队列中PAH诊断时的临床特征和随后的8年死亡率。

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，检测队列中的患者肺血管疾病进展较轻，这表现为更多患者处于纽约心脏协会I级和II级，平均肺动脉压和肺血管阻力指数较低，心输出量较高。检测队列中的患者接受利尿剂和华法林治疗的可能性较小，但两个队列在接受PAH特异性治疗方面没有差异。常规实践队列中的1年、3年、5年和8年生存率分别为75%、31%、25%和17%，而检测队列中的相应生存率分别为100%、81%、73%和64%（P = 0.0037）。