Supplemental Digital Content is available in the text. Pulmonary arterial hypertension is a serious complication of systemic lupus erythematosus. It is characterized by increased right ventricular afterload which mainly comprises pulmonary arterial compliance (PAC) and pulmonary vascular resistance. The role of PAC in predicting the outcome of systemic lupus erythematosus-associated pulmonary arterial hypertension has not been investigated yet. Between February 2012 to December 2016, 120 consecutive patients diagnosed with systemic lupus erythematosus-associated pulmonary arterial hypertension based on right heart catheterization were enrolled, prospectively. Baseline clinical characteristics and hemodynamic assessment were analyzed. Baseline right ventricular afterload was stratified according to the PAC and pulmonary vascular resistance. The end point was a composite of all-cause mortality and clinical worsening. Among them, end points occurred in 49 (41%) patients after 15 months (interquartile range, 8.5–24.0). Patients with a PAC <1.39 mL/mm Hg had a 3.09-fold higher risk (95% CI, 1.54–6.20, P=0.001) of the end point events than the patients with a PAC ≥1.39 mL/mm Hg. Multivariable Cox regression analysis showed that stratified right ventricular afterload was the only independent predictor for the end point (hazard ratio, 2.009 [95% CI, 1.390–2.904], P<0.001). A 3-group prediction risk was created. The patients with the highest right ventricular afterload (PAC <1.39 mL/mm Hg and pulmonary vascular resistance ≥10.3Wood Unit) had the highest risk (χ2, 6.10; P<0.014) of experiencing the end point. Our results suggest that PAC is a good predictor of mortality and clinical worsening in systemic lupus erythematosus-associated pulmonary arterial hypertension. PAC, in addition to pulmonary vascular resistance, may be an attractive tool for screening high-risk populations in these patients.

中文翻译：

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系统性红斑狼疮合并肺动脉高压患者肺动脉顺应性的预测价值

补充数字内容在文本中可用。肺动脉高压是系统性红斑狼疮的严重并发症。其特征是右心室后负荷增加，主要包括肺动脉顺应性（PAC）和肺血管阻力。PAC 在预测系统性红斑狼疮相关肺动脉高压结果中的作用尚未得到研究。2012 年 2 月至 2016 年 12 月期间，前瞻性地招募了 120 名基于右心导管插入术诊断为系统性红斑狼疮相关肺动脉高压的连续患者。分析了基线临床特征和血流动力学评估。根据 PAC 和肺血管阻力对基线右心室后负荷进行分层。终点是全因死亡率和临床恶化的复合终点。其中，49 名 (41%) 患者在 15 个月后出现终点（四分位距，8.5-24.0）。PAC <1.39 mL/mm Hg 患者的终点事件风险比 PAC ≥1.39 mL/mm Hg 患者高 3.09 倍（95% CI，1.54–6.20，P=0.001）。多变量 Cox 回归分析显示分层右心室后负荷是终点的唯一独立预测因子（风险比，2.009 [95% CI，1.390–2.904]，P<0.001）。创建了 3 组预测风险。右心室后负荷最高（PAC <1.39 mL/mm Hg 和肺血管阻力≥10.3Wood Unit）的患者经历终点的风险最高（χ2，6.10；P<0.014）。我们的结果表明，PAC 是系统性红斑狼疮相关肺动脉高压死亡率和临床恶化的良好预测指标。除了肺血管阻力外，PAC 可能是筛查这些患者高危人群的有吸引力的工具。