Fulminant Systemic Lupus Erythematosus Associated with Thrombotic Thrombocytopenic Purpura

We present a case of fulminant systemic lupus erythematosus (SLE) associated with thrombotic thrombocytopenic purpura (TTP) involving multiple organ failure. A 28-year-old woman diagnosed with SLE and treated with prednisolone for 6 months suddenly underwent severe heart failure and loss of consciousness. Laboratory tests showed severe anemia, thrombocytopenia, renal failure, and liver dysfunction. An extensive series of imaging examinations showed cerebral infarction, myocarditis, splenic infarction, and cystitis. Severe bleeding tendency caused hemorrhagic ascitis, hemorrhagic pleural effusion, hematuria, and systemic subcutaneous hemorrhage, and her general condition deteriorated. The patient was diagnosed with fulminant SLE associated with TTP and received intensive care including continuous hemodialysis, mechanical ventilation, and massive transfusion. One course of high-dose intravenous methylprednisolone and 5 series of plasma exchange (PE) suppressed SLE activity, and her general condition and consciousness gradually recovered. Thrombocytopenia was not controlled by these therapies. After using antiplatelet drugs, platelet count elevated dramatically. TTP is an unusual complication of SLE, and the treatment for fulminant SLE associated with TTP is not well known. In our case, combined treatment with PE, methylprednisolone, and antiplatelet drugs was effective for recovery.