The Prognosis and Management of Reclassified Systemic Lupus Erythematosus Associated Pulmonary Arterial Hypertension According to 2022 ESC/ERS Guidelines

Abstract

Abstract Background and Aims: The 2022 ESC/ERS guideline has recently revised the haemodynamic 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 reclassified by the new haemodynamic 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 analyze 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 (fulfilling the criteria of 2022 ESC/ERS guideline [mean pulmonary arterial pressure (mPAP) ≥ 21mmHg and pulmonary vascular resistance (PVR) > 2WU] but not the previous haemodynamic criteria [mPAP ≥ 25mmHg and PVR > 3WU]) according to the guideline and 14 were defined as partially remitted patients (mPAP 21–24 mmHg and PVR < 2 WU). The prognosis were compared among mild SLE-PAH, partially remitted SLE-PAH and conventional SLE-PAH patients (mPAP ≥ 25mmHg and PVR > 3WU). Besides, the effectiveness of pulmonary arterial hypertension (PAH) targeted 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, and the mild SLE-PAH patients had similar prognosis compared to partially remitted patients. Among the mild SLE-PAH patients, 4 did not receive PAH targeted therapy of PAH and had similar prognosis with patients not receiving targeted therapy. Besides, nearly all of the reclassified SLE-PAH patients received intensive immunosuppressive therapy (IIT). Conclusions This study supports the revised haemodynamic definition of SLE-PAH in the 2022 ESC/ERS guideline. Those reclassified mild and partially remitted SLE-PAH patients had better prognosis, demonstrating the possibility and significance of early diagnosis and intervention for SLE-PAH. This study also suggested that IIT against SLE might be sufficient for those reclassified SLE-PAH patients.