The prognosis and management of reclassified systemic lupus erythematosus associated pulmonary arterial hypertension according to 2022 ESC/ERS guidelines

IF 4.9 2区 医学 Q1 Medicine Arthritis Research & Therapy Pub Date : 2024-05-27 DOI:10.1186/s13075-024-03338-1
Yutong Li, Junyan Qian, Xingbei Dong, Jiuliang Zhao, Qian Wang, Yanhong Wang, Xiaofeng Zeng, Zhuang Tian, Mengtao Li
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Abstract

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. 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. 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. 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.
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根据 2022 年 ESC/ERS 指南重新分类的系统性红斑狼疮相关肺动脉高压的预后与管理
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液动力学定义。然而,目前有关系统性红斑狼疮相关性肺动脉高压(SLE-PAH)患者的预后和治疗的研究还很有限。本研究旨在分析新近被重新分类的系统性红斑狼疮相关性肺动脉高压患者的预后,并为治疗策略提供建议。这项回顾性研究分析了2011年至2023年期间在北京协和医院就诊的236名SLE-PAH患者的病历,其中22名患者被重新分类为轻度SLE-PAH(平均肺动脉压(mPAP)为21-24 mmHg、肺血管阻力(PVR)为 2-3 WU,PAWP ≤ 15 mmHg),14 名患者被定义为未分类的 SLE-PAH 患者(mPAP 为 21-24 mmHg,PVR ≤ 2 WU)。对轻度 SLE-PAH、未分类 SLE-PAH 和常规 SLE-PAH 患者(mPAP ≥ 25 mmHg 和 PVR > 3 WU)的预后进行了比较。此外,还对轻度系统性红斑狼疮-PAH 患者的肺动脉高压(PAH)特异性治疗效果进行了评估。与传统的系统性红斑狼疮-PAH患者相比,轻度系统性红斑狼疮-PAH患者的无进展时间明显更长。在轻度系统性红斑狼疮-PAH患者中,有4人未接受PAH特异性治疗,其预后与未接受特异性治疗的患者相似。这项研究支持2022年ESC/ERS指南中修订后的SLE-PAH血液动力学定义。那些轻度和未分类的系统性红斑狼疮-PAH 患者预后较好,这说明了早期诊断和干预系统性红斑狼疮-PAH 的可能性和意义。这项研究还提出了一个假设,即对于那些重新分类的系统性红斑狼疮-PAH 患者,针对系统性红斑狼疮的 IIT 可能就足够了。
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来源期刊
CiteScore
8.60
自引率
2.00%
发文量
261
审稿时长
14 weeks
期刊介绍: Established in 1999, Arthritis Research and Therapy is an international, open access, peer-reviewed journal, publishing original articles in the area of musculoskeletal research and therapy as well as, reviews, commentaries and reports. A major focus of the journal is on the immunologic processes leading to inflammation, damage and repair as they relate to autoimmune rheumatic and musculoskeletal conditions, and which inform the translation of this knowledge into advances in clinical care. Original basic, translational and clinical research is considered for publication along with results of early and late phase therapeutic trials, especially as they pertain to the underpinning science that informs clinical observations in interventional studies.
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