Cases of catastrophic anti-phospholipid syndrome in systemic lupus erythematosus: An experience. (March 2022)

• Record Type: Journal Article
• Authors: Hegde, Arun; Dorji, Thinley; Asturkar, Vikram; Yangzom, Sonam; Bhanu, Kovilapu Uday
• Is Part Of: SAGE open medical case reports. Volume 10(2022)
• Publication Date: 2022-03
• Subjects: Asherson's syndrome -- arterial thrombosis -- venous thrombosis -- emergency medicine -- lupus anti-coagulant -- anti-cardiolipin antibody -- anti-β2-glycoprotein-I antibody; Clinical medicine -- Case studies -- Periodicals; 616.09
• DOI: 10.1177/2050313X221085099
• Languages: English
• ISSNs: 2050-313X

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.