系统性硬皮病患者肺动脉高压的早期检测筛查：一项长期结局的系统评价和荟萃分析。
Screening for the early detection of pulmonary arterial hypertension in patients with systemic sclerosis: A systematic review and meta-analysis of long-term outcomes.
机构信息
The University of Melbourne, Victoria, Australia; The University of Melbourne, St. Vincent's Hospital, Level 3, 35 Victoria Pde, Fitzroy, Melbourne, Victoria 3065, Australia.
Rheumatology Department, The Royal Adelaide Hospital, Adelaide, Australia; Discipline of Medicine, The University of Adelaide, Adelaide, Australia.
出版信息
Semin Arthritis Rheum. 2021 Jun;51(3):495-512. doi: 10.1016/j.semarthrit.2021.03.011. Epub 2021 Apr 4.
BACKGROUND
Systemic sclerosis (scleroderma, SSc) is a chronic multisystem autoimmune disease characterised by fibrosis of the skin and internal organs and vasculopathy. One of the major contributors to mortality in patients with SSc is pulmonary arterial hypertension (PAH). International recommendations advise annual screening for the early detection of PAH in asymptomatic patients with SSc.
OBJECTIVES
To evaluate by systematic review current measures employed for screening for PAH. To summarise by meta-analysis the current evidence for long-term outcomes of screening for PAH in SSc.
METHODS
Manuscripts published until 12th March 2019 were identified through searching Medline, Embase and Cochrane Central Register of Controlled Trials and Database of Systematic Reviews. Eligible studies included abstracts or full reports investigating patients with SSc undergoing screening by any protocol to detect PAH. Risk of bias was assessed with reference to the QUADAS-2 tool.
RESULTS
The review resulted in 580 unique citations with 15 manuscripts included for final systematic review of screening methods, and six for meta-analysis. The systematic review demonstrated that there are varying protocols for screening for PAH. Screened populations were reported to have better risk stratification parameters at PAH diagnosis. Meta-analysis showed improved survival in patients with SSc-PAH diagnosed as a result of screening. There were trends towards having better risk stratification parameters at PAH diagnosis in those screened, although not all of these were statistically significant.
LIMITATIONS
There are no randomised controlled trials of screening for PAH in patients with SSc and the evidence presented in this review is derived from publications of registry data, cross-sectional and cohort studies.
CONCLUSIONS
This review demonstrates long-term benefit through the systematic screening of patients with SSc of varying disease duration for the early detection of PAH. Screened cohorts had improved survival, and were more likely to have better prognostic factors at the time of diagnosis with PAH.
背景
系统性硬化症（硬皮病，SSc）是一种慢性多系统自身免疫性疾病，其特征为皮肤和内脏器官纤维化以及血管病变。导致 SSc 患者死亡的主要原因之一是肺动脉高压（PAH）。国际建议建议每年对无症状的 SSc 患者进行 PAH 筛查，以早期发现 PAH。
目的
通过系统评价评估当前用于筛查 PAH 的方法。通过荟萃分析总结目前用于筛查 SSc 中 PAH 的长期结果的证据。
方法
通过搜索 Medline、Embase 和 Cochrane 对照试验中心注册库以及系统评价数据库，确定截至 2019 年 3 月 12 日发表的手稿。合格的研究包括调查接受任何筛查协议以检测 PAH 的 SSc 患者的摘要或完整报告。使用 QUADAS-2 工具评估偏倚风险。
结果
该综述共产生了 580 个独特的引文，其中 15 篇手稿纳入最终的筛查方法系统评价，6 篇纳入荟萃分析。系统评价表明，目前有多种用于筛查 PAH 的方案。筛查人群在 PAH 诊断时具有更好的风险分层参数。荟萃分析显示，由于筛查而诊断为 SSc-PAH 的患者的生存率有所提高。尽管并非所有这些都具有统计学意义，但在接受筛查的患者中，PAH 诊断时的风险分层参数也有改善的趋势。
局限性
目前没有对 SSc 患者进行 PAH 筛查的随机对照试验，本综述中提出的证据来自登记数据、横断面和队列研究的出版物。
结论
本综述表明，通过对不同疾病持续时间的 SSc 患者进行系统性筛查，可以早期发现 PAH，从而获得长期收益。筛查队列的生存率有所提高，并且在诊断为 PAH 时更有可能具有更好的预后因素。
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