Lupus Aortitis Leading to Aneurysmal Dilatation in the Aortic Root and Ascending Aorta

Abstract

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.