摘要 目的：肺动脉高压是系统性红斑狼疮少见而严重的临床表现，可最终导致死亡。系统性红斑狼疮相关肺动脉高压的生存预测因素的识别对于适当病情监测、治疗时机及肺移植治疗都是必要的。本项研究的主要目的是通过文献复习来识别系统性红斑狼疮相关肺动脉高压的生存预测因素。本研究也对预后研究的方法学质量进行了评估。

方法：本研究为评估系统性红斑狼疮相关肺动脉高压的预后因素进行了系统性文献复习，对Medline, EMBASE, CINAHL等文献系统及Cochrane中央注册中心的对照试验系统进行了检索。本研究由两位独立评审员采取两种标准化方法来选取预后因素。方法学质量的评估采取了验证质量指数。

结果：研究评估了有375次引用的23项观察性研究。平均肺动脉压升高的程度、雷诺现象、血小板减少、肺动脉丛状损伤、感染、血栓形成、妊娠、肺血管炎及抗心磷脂抗体与生存率下降相关。而狼疮疾病活动度、肾炎、中枢系统疾病与生存无关。研究样本量小，且研究方法学质量是多变的。

结论：本研究总结了系统性红斑狼疮相关肺动脉高压患者生存率下降的相关因素。样本量小且多变的方法学质量阻碍了确定性结论的得出。本研究为未来大规模队列研究奠定了基础。

附原文: Abstract OBJECTIVE: Pulmonary hypertension (PH) is a rare but severe manifestation of systemic lupus erythematosus (SLE) that can ultimately result in death. The identification of factors that prognosticate survival in SLE-PH is necessary for appropriate monitoring, timing of therapeutics and lung transplantation. The primary objective of this study was to identify prognostic factors for survival in SLE-PH through review of the literature. The methodological quality of the prognostic studies was also evaluated. METHODS: A systematic review of the literature was performed to identify studies evaluating prognostic factors for survival in SLE-PH. Medline, EMBASE, CINAHL, and Cochrane Central Registry of Controlled Trials (inception - week 2 2010) were searched. A standardized abstraction form. was used by two independent reviewers to extract prognostic factors. Methodological quality was evaluated using a validated quality index. RESULTS: Twenty-three observational studies from 375 citations were evaluated. Elevated mean pulmonary artery pressure, Raynaud's phenomenon, thrombocytopenia, plexiform. lesion, infection, thrombosis, pregnancy, pulmonary vasculitis and anticardiolipin antibodies were associated with decreased survival. Lupus disease activity, nephritis and central nervous system disease were not associated with survival. The sample sizes were small and methodological quality of the studies was variable. CONCLUSION: This study summarizes factors that may be associated with decreased survival in SLE-PH. The small sample sizes and variable methodological quality preclude definitive conclusions. This study provides the groundwork for further research using large cohorts.

引自: Chow SL, Chandran V, Fazelzad R, Prognostic factors for survival in systemic lupus erythematosus associated pulmonary hypertension.Lupus. 2012 Apr;21(4):353-64