合并肺动脉高压的狼疮患者生存的预后因素

        摘要（加拿大）  目的 肺动脉高压是狼疮的少见但严重的表现，可最终导致死亡。确定合并肺动脉高压的狼疮患者的预后生存因素有助于监测、及时治疗和肺移植。本研究的主要目的是通过文献复习，来证实狼疮肺动脉高压患者的生存预后因素，同时评价文献对预后方法学研究的质量。

        方法:通过检索Medline、 EMBASE、CINAHL和Cochrane中心注册性对照试验(截止到2010年) ，进行文献系统综述来证实SLE-PAH生存的预后因素。2个独立研究者用一个标准表格来提取预后因素，采用有效质量指数来评价方法学质量。

        结果: 从375条引文中提取23项观察研究进行评价发现，与生存率降低相关的因素包括肺动脉压平均增高幅度、雷诺现象、血小板减少、丛状病变、感染、血栓形成、怀孕、肺血管炎和抗心磷脂抗体，而狼疮病情活动性、中枢神经系统病变和肾炎与生存率无关，样本量少和研究的方法学质量各不相同。

        结论 本研究总结了可能与SLE-PAH生存率降低有关的因素，但样本量少和方法学质量不相同尚不能得出确切的结论，本研究为未来进行大样本队列研究打下了基础。

        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, Johnson SR. Prognostic factors for survival in systemic lupus erythematosus associated pulmonary hypertension. Lupus. 2011 Nov 29. [Epub ahead of print]