Journal of Rare Disorders: Diagnosis & Therapy

SLE Dissecting the Human Body

Introduction: Aortic dissection (AD) is a rare but life threatening condition, with a cumulative mortality rate of 68% within 48 hours. Systemic lupus erythematosus (SLE) has classically been associated with heterogeneous multi-system involvement, but SLE causing AD is not well recognized. In this case, we will describe a patient with a history of SLE that presented with unique vascular complications involving major vessels.

Case description: A 35-year-old female presented to emergency department with one day history of acute onset retrosternal chest pain at rest and shortness of breath. Patient had a history of SLE, lupus nephritis on hemodialysis, hypertension and chronic brachiocephalic artery dissection with extension to right carotid artery that had been managed conservatively. Her medications included hydroxychloroquine and prednisone. Further evaluation showed elevated troponins to 19 ng/mL and bilateral pulmonary edema. Echocardiography showed normal left ventricular function, new severe aortic insufficiency with possible ascending AD. Computed tomography angiogram of the aorta was inconclusive for AD. Due to high clinical suspicion, transesophageal echocardiogram was done, and showed a ring-like flap protruding into the aortic root in the vicinity of both coronary ostia, confirming ascending AD. Patient emergently underwent surgical repair of type A AD, and was found to have circumferential tear at the sinotubular junction. She had a stable hospital course.

Discussion: Review of literature reveals that AD in SLE patients is extremely rare, and only 22 cases were reported over recent years. Common features in such patients include young age, presence of systemic disease, hypertension and chronic corticosteroids use. Long-term outcomes After AD repair are still unfavorable, and patients need careful monitoring. Annual linearized mortality rate After Valve-sparing AD repair is 4.7% per year.

Author(s):

Saed Alnaimat, Emad Hakemi, Nadia El Hangouche and Victor Pelaez

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