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Abstract：
OBJECTIVE: The 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guideline has recently revised the hemodynamic definition of pulmonary arterial hypertension. However, there is currently limited research on the prognosis and treatment of system lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH) patients that have been reclassified by the new hemodynamic definition. This study aims to analyze the prognosis of newly reclassified SLE-PAH patients and provide recommendations for the management strategy.
METHODS: This retrospective study analyzed records of 236 SLE-PAH patients who visited Peking Union Medical College Hospital (PUMCH) from 2011 to 2023, among whom 22 patients were reclassified into mild SLE-PAH (mean pulmonary arterial pressure (mPAP) of 21-24 mmHg, pulmonary vascular resistance (PVR) of 2-3 WU, and PAWP ≤ 15 mmHg) according to the guidelines and 14 were defined as unclassified SLE-PAH patients (mPAP 21-24 mmHg and PVR ≤ 2 WU). The prognosis was compared among mild SLE-PAH, unclassified SLE-PH, and conventional SLE-PAH patients (mPAP ≥ 25 mmHg and PVR > 3WU). Besides, the effectiveness of pulmonary arterial hypertension (PAH)-specific therapy was evaluated in mild SLE-PAH patients.
RESULTS: Those mild SLE-PAH patients had significantly longer progression-free time than the conventional SLE-PAH patients. Among the mild SLE-PAH patients, 4 did not receive PAH-specific therapy and had a similar prognosis as patients not receiving specific therapy.
CONCLUSIONS: This study supports the revised hemodynamic definition of SLE-PAH in the 2022 ESC/ERS guideline. Those mild and unclassified SLE-PH patients had a better prognosis, demonstrating the possibility and significance of early diagnosis and intervention for SLE-PAH. This study also proposed a hypothesis that IIT against SLE might be sufficient for those reclassified SLE-PAH patients.
METHODS: This retrospective study analyzed records of 236 SLE-PAH patients who visited Peking Union Medical College Hospital (PUMCH) from 2011 to 2023, among whom 22 patients were reclassified into mild SLE-PAH (mean pulmonary arterial pressure (mPAP) of 21-24 mmHg, pulmonary vascular resistance (PVR) of 2-3 WU, and PAWP ≤ 15 mmHg) according to the guidelines and 14 were defined as unclassified SLE-PAH patients (mPAP 21-24 mmHg and PVR ≤ 2 WU). The prognosis was compared among mild SLE-PAH, unclassified SLE-PH, and conventional SLE-PAH patients (mPAP ≥ 25 mmHg and PVR > 3WU). Besides, the effectiveness of pulmonary arterial hypertension (PAH)-specific therapy was evaluated in mild SLE-PAH patients.
RESULTS: Those mild SLE-PAH patients had significantly longer progression-free time than the conventional SLE-PAH patients. Among the mild SLE-PAH patients, 4 did not receive PAH-specific therapy and had a similar prognosis as patients not receiving specific therapy.
CONCLUSIONS: This study supports the revised hemodynamic definition of SLE-PAH in the 2022 ESC/ERS guideline. Those mild and unclassified SLE-PH patients had a better prognosis, demonstrating the possibility and significance of early diagnosis and intervention for SLE-PAH. This study also proposed a hypothesis that IIT against SLE might be sufficient for those reclassified SLE-PAH patients.
摘要：
目的：2022年欧洲心脏病学会/欧洲呼吸学会（ESC/ERS）指南最近修订了肺动脉高压的血流动力学定义。然而,目前,关于系统性红斑狼疮相关肺动脉高压(SLE-PAH)患者的预后和治疗的研究有限,这些患者已通过新的血流动力学定义重新分类.本研究旨在分析新重新分类的SLE-PAH患者的预后，并为管理策略提供建议。
方法：这项回顾性研究分析了2011年至2023年在北京协和医院就诊的236例SLE-PAH患者的记录，其中22例患者被重新分类为轻度SLE-PAH（平均肺动脉压（mPAP）为21-24mmHg，2-3WU的肺血管阻力（PVR），和PAWP≤15mmHg）根据指南，14例被定义为未分类的SLE-PAH患者（mPAP21-24mmHg和PVR≤2WU）。比较轻度SLE-PAH的预后，未分类的SLE-PH,和常规SLE-PAH患者（mPAP≥25mmHg且PVR>3WU）。此外,在轻度SLE-PAH患者中评估了肺动脉高压(PAH)特异性治疗的有效性.
结果：轻度SLE-PAH患者的无进展时间明显长于常规SLE-PAH患者。在轻度SLE-PAH患者中，4例患者未接受PAH特异性治疗，其预后与未接受特异性治疗的患者相似。
结论：本研究支持2022年ESC/ERS指南中SLE-PAH的血流动力学定义。轻度和未分类的SLE-PH患者预后较好，证明SLE-PAH早期诊断和干预的可能性和意义。这项研究还提出了一个假设，即针对SLE的IIT对于那些重新分类的SLE-PAH患者可能是足够的。
方法：这项回顾性研究分析了2011年至2023年在北京协和医院就诊的236例SLE-PAH患者的记录，其中22例患者被重新分类为轻度SLE-PAH（平均肺动脉压（mPAP）为21-24mmHg，2-3WU的肺血管阻力（PVR），和PAWP≤15mmHg）根据指南，14例被定义为未分类的SLE-PAH患者（mPAP21-24mmHg和PVR≤2WU）。比较轻度SLE-PAH的预后，未分类的SLE-PH,和常规SLE-PAH患者（mPAP≥25mmHg且PVR>3WU）。此外,在轻度SLE-PAH患者中评估了肺动脉高压(PAH)特异性治疗的有效性.
结果：轻度SLE-PAH患者的无进展时间明显长于常规SLE-PAH患者。在轻度SLE-PAH患者中，4例患者未接受PAH特异性治疗，其预后与未接受特异性治疗的患者相似。
结论：本研究支持2022年ESC/ERS指南中SLE-PAH的血流动力学定义。轻度和未分类的SLE-PH患者预后较好，证明SLE-PAH早期诊断和干预的可能性和意义。这项研究还提出了一个假设，即针对SLE的IIT对于那些重新分类的SLE-PAH患者可能是足够的。
