Abstract
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, and haematologic manifestations are part of its spectrum. Herein, we report a case of a patient with a long-standing diagnosis of SLE, presenting with thrombotic thrombocytopenic purpura (TTP) and acute renal failure, without co-existent clinical and laboratory markers of disease activity, causing diagnostic questions. A short literature review concerning TTP and SLE is also presented. TTP is a rare syndrome of thrombotic microangiopathy, which represents a medical urgency and carries significant morbidity and mortality if left untreated. SLE has been correlated with the occurrence of TTP, often with atypical presentation and worse prognosis.