Kiani Rabia, Siddiqui Muhammad Danial, Tantoush Hamza
Department of Internal Medicine, University of South Dakota, Vermillion, SD, USA.
Case Rep Rheumatol. 2020 May 15;2020:6014572. doi: 10.1155/2020/6014572. eCollection 2020.
Severe pulmonary artery hypertension (PAH) is a rare initial presentation of systemic lupus erythematosus (SLE). SLE associated with PAH carries worse prognosis that isolated SLE. However, there has been improvement in mortality of the patients in the recent years owing to newer treatment options available. Early recognition remains of prime importance. We present here a case of young female who presented with severe pulmonary hypertension with right heart failure leading to cardiogenic shock and was found to have SLE. She was started on appropriate treatment; however, given the severity of her illness, the patient did not survive. This case highlights the importance of early recognition and prompt treatment of SLE-associated PAH, which might improve the survival rate in the patients.
重度肺动脉高压（PAH）是系统性红斑狼疮（SLE）罕见的初始表现。与PAH相关的SLE预后比单纯SLE更差。然而，由于有了新的治疗选择，近年来患者的死亡率有所改善。早期识别仍然至关重要。我们在此介绍一例年轻女性病例，该患者表现为重度肺动脉高压伴右心衰竭，导致心源性休克，最终被诊断为SLE。她开始接受适当治疗；然而，鉴于病情严重，患者未能存活。该病例强调了早期识别和及时治疗SLE相关PAH的重要性，这可能会提高患者的生存率。
