Lee Cody, Elwing Jean
Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA.
Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA.
SAGE Open Med Case Rep. 2017 May 4;5:2050313X17707153. doi: 10.1177/2050313X17707153. eCollection 2017.
The pulmonary vascular targeted treatment for systemic lupus erythematosus-associated pulmonary arterial hypertension is similar to other connective tissue disease-associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject. Our objective is to highlight the importance of early immunosuppression in systemic lupus erythematosus-associated pulmonary arterial hypertension and the role of pulse dose steroids in management.
This case describes a 23-year-old woman who presented with pulmonary arterial hypertension diagnosed by right heart catheterization with mean pulmonary artery pressure of 74 mmHg, pulmonary capillary wedge pressure of 12 mmHg, and a pulmonary vascular resistance of 1908 dyne s cm. Due to the aggressive nature of her disease, she declined despite management with epoprostenol and sildenafil. Because of coexisting systemic lupus erythematosus with hemolytic anemia and worsening pulmonary arterial hypertension, intensive immunosuppressive therapy with pulse dose steroids was initiated.
Shortly after initiation of pulse dose steroids and maintenance immunosuppression, she had a dramatic symptomatic and hemodynamic response with a decrease in her pulmonary vascular resistance from 1908 to 136 dyne sec cm and improvement in her mean pulmonary artery pressure from 74 to 27 mmHg on repeat right heart catheterization.
Early immunosuppression is important to consider in those with systemic lupus erythematosus-associated pulmonary arterial hypertension. Limited studies are available, but most have focused on the use of cyclophosphamide. Pulse dose steroids may be a potentially less toxic but equally effective manner to aid in the treatment of systemic lupus erythematosus-pulmonary arterial hypertension when intensive immunosuppression is being considered.
系统性红斑狼疮相关肺动脉高压的肺血管靶向治疗与其他结缔组织病相关肺动脉高压相似。此外，免疫抑制在整体治疗中似乎也发挥着作用。然而，鉴于缺乏关于该主题的随机对照试验，目前尚不清楚最佳免疫抑制方案以及哪些患者会对治疗产生反应。我们的目的是强调早期免疫抑制在系统性红斑狼疮相关肺动脉高压中的重要性以及冲击剂量类固醇在治疗中的作用。
本病例描述了一名23岁女性，经右心导管检查诊断为肺动脉高压，平均肺动脉压为74mmHg，肺毛细血管楔压为12mmHg，肺血管阻力为1908达因·秒/厘米。由于其病情严重，尽管使用依前列醇和西地那非进行治疗，她的病情仍在进展。由于同时存在系统性红斑狼疮伴溶血性贫血且肺动脉高压恶化，遂开始采用冲击剂量类固醇进行强化免疫抑制治疗。
在开始冲击剂量类固醇治疗并维持免疫抑制后不久，她出现了显著的症状和血流动力学反应，重复右心导管检查显示其肺血管阻力从1908降至136达因·秒/厘米，平均肺动脉压从74降至27mmHg。
对于系统性红斑狼疮相关肺动脉高压患者，应考虑早期免疫抑制。现有研究有限，且大多数集中在环磷酰胺的使用上。当考虑进行强化免疫抑制时，冲击剂量类固醇可能是一种毒性较低但同样有效的辅助治疗系统性红斑狼疮相关肺动脉高压的方法。
