Systemic Lupus Erythematosus-Associated Thrombotic Thrombocytopenic Purpura: A Case Report

Abstract

Systemic lupus erythematosus-associated thrombotic thrombocytopenic purpura (SLE-TTP) is a rare but life-threatening condition that requires prompt recognition and treatment. We report a case of a patient with systemic lupus erythematosus (SLE) who presented with encephalopathy and was subsequently diagnosed with thrombotic thrombocytopenic purpura (TTP) based on ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13) deficiency. The patient was successfully treated with plasmapheresis. A 42-year-old woman with a history of SLE presented with febrile encephalopathy and was admitted to the intensive care unit (ICU). Laboratory evaluation revealed microangiopathic hemolytic anemia and severe thrombocytopenia. MRI showed leptomeningeal enhancement and white matter changes suggestive of neuro-lupus. However, ADAMTS13 activity was <1% with detectable anti-ADAMTS13 antibodies, confirming the diagnosis of TTP. The patient received four sessions of plasmapheresis, high-dose corticosteroids, rituximab, cyclophosphamide, and supportive care. Following a steady improvement in consciousness, she was transferred to the internal medicine ward on day 17, with marked clinical and laboratory recovery. This case highlights the importance of considering TPP in SLE patients presenting with acute neurological symptoms. Early recognition, prompt initiation of plasmapheresis, and immunosuppressive therapy can lead to favorable clinical and biological outcomes.