Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry
Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry
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DOI：
10.3899/jrheum.180018
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发表时间：
2019-02-01
影响因子：
3.9
通讯作者：
Steen, Virginia D.
中科院分区：
文献类型：
--
作者：
Hsu, Vivien M.;Chung, Lorinda;Steen, Virginia D.
Objective.我们试图确定有肺动脉高压（PH）风险的患者的死亡率和心肺住院的预测因素，并纳入PHAROS，这是一项前瞻性队列研究，旨在调查系统性硬化症（SSc）中PH的自然病史。PH的高危人群由以下入选标准定义：超声心动图收缩期肺动脉压> 40 mmHg，或DLCO < 55%预测值或通过肺功能测试测量的%用力肺活量/%DLCO的比值> 1.6。基线临床指标被评估为2005年至2014年期间住院和死亡的预测因素。考克斯比例风险模型在PH发作或最后一次研究访视时删失，并根据年龄、性别、种族和疾病持续时间进行调整。在236例中位随访4年（范围0.4-8.5年）的高危受试者中，35例在进入PHAROS后发生PH（重新分类为PH组）。在高危组中，较高的死亡率与男性、低% DLCO、运动氧饱和度下降、贫血、异常呼吸困难评分和基线心包积液密切相关。心肺住院的风险与呼吸困难和心包积液的增加相关，尽管DLCO < 50%的PH患者心肺住院的风险最高。有PH风险的SSc患者预后不良的风险因素与其他SSc-PH和SSc-肺动脉高压患者相似，包括男性、DLCO <50%、运动氧饱和度下降和心包积液。这组患者应接受右心导管检查，如果确认PH，应接受适当的干预。
Objective. We sought to identify predictors of mortality and cardiopulmonary hospitalizations in patients at risk for pulmonary hypertension (PH) and enrolled in PHAROS, a prospective cohort study to investigate the natural history of PH in systemic sclerosis (SSc).Methods. The at-risk population for PH was defined by the following entry criteria: echocardiogram systolic pulmonary arterial pressure > 40 mmHg, or DLCO < 55% predicted or ratio of % forced vital capacity/%DLCO > 1.6, measured by pulmonary function testing. Baseline clinical measures were evaluated as predictors of hospitalization and death between 2005 and 2014. Cox proportional hazards models were censored at date of PH onset or latest study visit and adjusted for age, sex, race, and disease duration.Results. Of the 236 at-risk subjects who were followed for a median of 4 years (range 0.4-8.5 yrs), 35 developed PH after entering PHAROS (reclassified as PH group). In the at-risk group, higher mortality was strongly associated with male sex, low % DLCO, exercise oxygen desaturation, anemia, abnormal dyspnea scores, and baseline pericardial effusion. Risks for cardiopulmonary hospitalization were associated with increased dyspnea and pericardial effusions, although PH patients with DLCO < 50% had the highest risk of cardiopulmonary hospitalizations.Conclusion. Risk factors for poor outcome in patients with SSc who are at risk for PH were similar to others with SSc-PH and SSc-pulmonary arterial hypertension, including male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions. This group should undergo right heart catheterization and receive appropriate intervention if PH is confirmed.