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

• Published in: SAGE open medical case reports Vol. 10; p. 2050313X221085099
• Main Authors: Hegde, Arun, Dorji, Thinley, Asturkar, Vikram, Yangzom, Sonam, Bhanu, Kovilapu Uday
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.03.2022; Sage Publications Ltd; SAGE Publishing
• Subjects: Case Report; Case reports; Lupus; Thrombosis; anti-β2-glycoprotein-I antibody; emergency medicine; lupus anti-coagulant; anti-cardiolipin antibody; Asherson’s syndrome; arterial thrombosis; venous thrombosis
• ISSN: 2050-313X; 2050-313X
• DOI: 10.1177/2050313X221085099

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.