Acute type A aortic dissection in a patient with systemic lupus erythematosus

The case of a 27-year-old Japanese woman with type A acute aortic dissection who had been diagnosed with systemic lupus erythematosus (SLE) is presented. The patient also had aortic regurgitation due to non-infective endocarditis and systemic hypertension, and had been maintained on steroid therapy for 15 years. Her twin sister was also diagnosed with SLE. The patient was admitted to emergency due to severe back pain. A chest x-ray showed enlargement of the upper mediastinum. Echocardiography revealed a thickened and deformed aortic valve with aortic regurgitation and dissection of the ascending aorta, but pericardial effusion was not found. Computed tomography demonstrated aortic dissection extending from the ascending aorta to the abdominal aorta. Graft replacement of the ascending aorta and proximal aortic arch was performed under hypothermic circulatory arrest with retrograde cerebral perfusion. The patient recovered uneventfully. Aortic dissection complicated with SLE is extremely rare, and this is only the 15th case reported in the English or Japanese literature.