Surgical Treatment of Acute Aortic Dissection in a Patient with SLE and Prior Antiphospholipid Syndrome on Therapy for over 30 Years: a Case Report

Background: In patients with systemic lupus erythematosus (SLE), lengthy treatment and long-term steroid use are the main risk factors for developing aortic aneurysms or aortic dissections. In patients with cardiac tamponade, hemodynamic collapse may lead to acute renal and hepatic failure.

Case Presentation: We report the successful treatment of a 55-year-old woman with SLE since the age of 21. She suddenly felt chest pain approximately 2 weeks before developing fever and vomiting and was admitted to our hospital. Initially, she had severe liver dysfunction and was admitted to the hepatology department, where treatment for fulminant hepatitis was initiated. However, computed tomography (CT) showed an acute aortic dissection (DeBakey type II) and severe bloody pericardial effusion. Therefore, we performed emergency pericardial drainage. Plasma exchange therapy was initiated as emergency aortic surgery was deemed impossible due to impaired liver function tests and coagulation. Ten days later, the patient developed peritonitis due to small bowel perforation, and laparotomy was performed for abscess drainage and perforation closure. She had received steroid pulse therapy at the age of 21. At 40 years of age, she developed deep vein thrombosis due to antiphospholipid antibodies and was prescribed prednisolone. She was ambulatory at 3 months after the onset of acute aortic dissection, and CT revealed a rapidly enlarging true aneurysm in the distal arch. We performed elective aortic surgery. Although there were no antiphospholipid antibodies, surgery could have led to a devastating antiphospholipid syndrome. Therefore, we decided to treat the patient with triple therapy. Methylprednisolone was intravenously administered intraoperatively and at 1 day postoperatively. The patient was discharged without complications after returning to her usual oral prednisolone regimen.

Conclusions: The patient described herein had a systemic circulatory failure due to cardiac tamponade, accompanied by liver failure. This condition is a significant cause of death in patients with aortic dissection-associated SLE and is extremely dangerous. However, multi-specialty intervention helped the patient recover, and she has been attending the outpatient clinic. Aortic surgery requiring hypothermia in SLE patients with antiphospholipid syndrome and a history of thrombocytopenia or thrombosis requires a multi-disciplinary treatment team, including cardiac surgeons and medical experts.