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

• Published in: Arthritis research & therapy Vol. 26; no. 1; pp. 109 - 8
• Main Authors: Li, Yutong, Qian, Junyan, Dong, Xingbei, Zhao, Jiuliang, Wang, Qian, Wang, Yanhong, Zeng, Xiaofeng, Tian, Zhuang, Li, Mengtao
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 27.05.2024; BioMed Central; BMC
• Subjects: Adult; Analysis; B cells; Care and treatment; Diagnosis; Evidence-based medicine; Female; Hemodynamics; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - physiopathology; Hypertension, Pulmonary - therapy; Lupus; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - diagnosis; Male; Medical colleges; Medical research; Medicine, Experimental; Middle Aged; Practice Guidelines as Topic - standards; Prognosis; Pulmonary Arterial Hypertension - diagnosis; Pulmonary Arterial Hypertension - etiology; Pulmonary Arterial Hypertension - physiopathology; Pulmonary hypertension; Retrospective Studies; Risk factors; Strategic planning (Business); System lupus erythematosus associated pulmonary arterial hypertension; Systemic lupus erythematosus; China; System lupus erythematosus associated pulmonary arterial hypertension; Hemodynamics
• ISSN: 1478-6362; 1478-6354; 1478-6362
• DOI: 10.1186/s13075-024-03338-1

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.