Cases of catastrophic anti-phospholipid syndrome in systemic lupus erythematosus: An experience

Abstract

Anti-phospholipid syndrome is an autoimmune disorder characterized by episodes of arterial and/or venous thrombosis and/or pregnancy morbidity in the presence of anti-phospholipid antibodies. Catastrophic anti-phospholipid syndrome is an accelerated form of the disease with rapid involvement of multiple organ systems often posing a diagnostic challenge. There is a paucity of literature on the myriad presentations of catastrophic anti-phospholipid syndrome owing to the orphan nature of the disease. We present three cases of catastrophic anti-phospholipid syndrome in patients with systemic lupus erythematosus that presented with episodes of thrombosis involving both arterial and venous systems and multisystem organ failure. Timely diagnoses were made based on a high index of suspicion and were managed with a combination of systemic glucocorticoids, cyclophosphamide, plasmapheresis, intravenous immunoglobulin and other supportive measures. However, despite providing the standard of care, we encountered a poor outcome in two of these patients, highlighting the high mortality associated with catastrophic anti-phospholipid syndrome.