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

Authors

  • Derek R. Brinster
  • John D. Grizzard
  • Alok Dash

DOI:

https://doi.org/10.1532/HSF98.20081123

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.

References

Braunwald E, Fauci AS, Kasper DL. 2005. Systemic lupus erythematosus. In: Kasper DL, Braunwald E, Hauser S, Longo D, Jameson JL, Fauci AS, eds. Harrison's principles of internal medicine. 16th ed. New York, NY: McGraw-Hill. p 1960-7.nChakravarty K, Scott DGI. 1992. Mycotic aneurysm of the aortic arch masquerading as systemic lupus erythematosus. Ann Rheum Dis 51:1079-81.nDoherty NE, Siegel RJ. 1985. Cardiovascular manifestations systemic lupus erythematosus. Am Heart J 110:1257-65.nGuard RW, Graham IG, Edmonds JP, Thomas AC. 1995. Aortitis with dissection complicating systemic lupus erythematosus. Pathology 27:224-8.nHachulla E, Beregi JP. 2001. Diagnosis of aortitis [in French]. J Mal Vasc 26:223-7.nMacLeod CB, Johnson D, Frable WJ. 1992. ‘Tree-barking’ of the ascending aorta: syphilis or systemic lupus erythematosus? Am J Clin Pathol 97:58-62.nOhara N, Miyata T, Kurata A, Oshiro H, Sato O, Shigematsu H. 2000. Ten years' experience of aortic aneurysm associated with systemic lupus erythematosus. Eur J Vasc Endovasc Surg 19:288-93.nSchur P, Wallace D. 2007. Overview of the therapy and prognosis of systemic lupus erythematosus in adults. UpToDate Web site. Available at: http://www.uptodate.com'>http://www.uptodate.comnSilver AS, Shao CY, Ginzler EM. 2006. Aortitis and aortic thrombus in systemic lupus erythematosus. Lupus 15:541-3.nStehbens WE, Delahunt B, Shirer WC, Naik DK. 1993. Aortic aneurysm in systemic lupus erythematosus. Histopathology 22:275-7.nTakagi H, Mori Y, Iwata H, et al. 2002. Nondissecting aneurysm of the thoracic aorta with arteritis in systemic lupus erythematosus. J Vasc Surg 35:801-4.nTavora F, Burke A. 2006. Review of isolated ascending aortitis: differential diagnosis, including syphilitic, Takayasu's and giant cell aortitis. Pathology 38:302-3.nWang J, French SW, Chuang CC, McPhaul L. 2000. Pathologic quiz case: an unusual complication of systemic lupus erythematosus. Arch Pathol Lab Med 124:324-6.n

Published

2009-04-20

How to Cite

Brinster, D. R., Grizzard, J. D., & Dash, A. (2009). Lupus Aortitis Leading to Aneurysmal Dilatation in the Aortic Root and Ascending Aorta. The Heart Surgery Forum, 12(2), E105-E108. https://doi.org/10.1532/HSF98.20081123

Issue

Section

Articles