IntroductionThrombotic thrombocytopenic purpura (TTP) is a rare blood disorder characterized by the formation of small blood clots throughout the body, resulting in a reduction in platelet count, known as thrombocytopenia. This condition can occur as an idiopathic condition or as a secondary complication of other diseases, such as systemic lupus erythematosus (SLE). The association between TTP and SLE is particularly worrisome due to its high mortality rate, ranging from 34 to 62.5% [1,2]. The overlapping clinical symptoms of TTP and SLE, including fatigue, rash, and fever, can make diagnosing challenging. However, early detection and treatment, especially through plasma exchange and immunosuppressive therapies, are crucial in improving patient outcomes.The morbidity and mortality risks associated with TTP and lupus nephritis are significant. TTP, when linked to autoimmune conditions like SLE, poses a higher mortality risk compared to idiopathic TTP. Thrombocytopenia, particularly with platelet counts below 18 × 10^9/L, is a significant risk factor for mortality in SLE patients experiencing thrombotic microangiopathy (TMA). In patients with SLE-TTP, renal involvement is less severe than in primary immune thrombotic thrombocytopenic purpura (iTTP), and mortality rates are significantly lower compared to those with primary iTTP.Thrombotic thrombocytopenic purpura occurs due to a deficiency in ADAMTS13, a von Willebrand factor- cleaving protease. This deficiency is often a result of autoantibodies targeting ADAMTS13 or, rarely, a mutation in the gene encoding ADAMTS13. The disease is more prevalent among women in the United States, with African Americans facing an eightfold increase in risk. Timely recognition and treatment are crucial, as severe ADAMTS13 deficiency (<10%) can lead to the aggregation of ultra-large von Willebrand factor multimers, resulting in uncontrolled platelet adhesion and the formation of disseminated microthrombi, causing organ ischemia. In this article we present a case of TTP with overlapping symptoms of lupus nephritis which posed a significant diagnostic challenge.