Brinster Derek R, Grizzard John D, Dash Alok
Thoracic Aortic Surgery Program, Division of Cardiothoracic Surgery, Virginia Commonwealth University Medical Center, Medical College of Virginia Campus, Richmond, Virginia 23298-0068, USA.
Heart Surg Forum. 2009 Apr;12(2):E105-8. doi: 10.1532/HSF98.20081123.
Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs, tissues, and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes. We describe the case of a 23-year-old African American woman with a history of recurrent pneumonias. Computed tomography, magnetic resonance imaging (MRI), and echocardiographic evaluations, as well as clinical and laboratory findings, indicated a diagnosis of SLE with inflammatory aortitis secondary to SLE vasculitis. A repeat MRI revealed a rapidly expanding aortic root and ascending aorta that required prompt operative repair. The ascending aorta and aortic root were replaced with a mechanical valved conduit, and a coronary artery bypass to the posterior descending artery was performed because of related erosion into the intima of the right coronary ostium. The patient has done well postoperatively. Aortitis and aortic aneurysms are an uncommon manifestation of SLE, and a literature search revealed an apparent association between aortic aneurysms and steroid medications for SLE. This case is the first report of aortitis resulting in a nondissecting aortic root aneurysm in an SLE patient without a history of steroid use, indicating that all SLE patients, including those without a history of steroid use, require screening for aortic disease to improve surgical outcomes and to prevent fatal complications.
系统性红斑狼疮(SLE)是一种自身免疫性疾病,其中器官、组织和细胞会受到组织结合自身抗体和免疫复合物介导的损伤。我们描述了一名患有复发性肺炎病史的23岁非裔美国女性的病例。计算机断层扫描、磁共振成像(MRI)和超声心动图评估,以及临床和实验室检查结果,表明诊断为SLE并伴有继发于SLE血管炎的炎症性主动脉炎。重复MRI显示主动脉根部和升主动脉迅速扩张,需要立即进行手术修复。用带机械瓣膜的管道替换升主动脉和主动脉根部,并因右冠状动脉开口内膜相关侵蚀而进行了后降支冠状动脉搭桥术。患者术后恢复良好。主动脉炎和主动脉瘤是SLE的罕见表现,文献检索显示主动脉瘤与SLE的类固醇药物之间存在明显关联。该病例是首例无类固醇使用史的SLE患者发生非夹层主动脉根部瘤的主动脉炎报告,表明所有SLE患者,包括无类固醇使用史的患者,都需要筛查主动脉疾病,以改善手术结果并预防致命并发症。