Rare Association of Antiphospholipid Antibody Syndrome, Systemic Lupus Erythematosus and Aortic Dissection: A Striking Presentation with Multi-Organ Failure?

Systemic lupus erythematosus is a chronic autoimmune disease with a wide variety of clinical presentations induced by different immunocomplexes and autoantibodies. Antiphospholipid antibody syndrome (APLAS) is a life-threatening clinical condition characterized by venous and arterial thromboses or pregnancy morbidity in the presence of persistent moderate/high levels of antiphospholipid antibodies. Aortic dissection is rarely associated with APLAS and always requires prompt diagnosis and early treatment. We report a rare case with a striking presentation. The patient developed multi-organ failure due to lethal aortic dissection and the obstruction of abdominal and thoracic branch vessels. LEARNING POINTS: Aortic dissection is a rare lethal clinical condition that always requires prompt diagnosis and early treatment.Signs of multi-organ ischaemia due to obstruction of abdominal and thoracic branch vessels should be kept in mind by clinicians.Venous thrombosis and medial wall necrosis in the aorta may be underlying complex pathophysiological mechanisms in patients with antiphospholipid antibody syndrome. © EFIM 2020.