Severe Lupus Nephritis with Thrombotic Microangiopathy: A Case Report

Abstract

ABSTRACT Thrombotic microangiopathy (TMA), a vascular lesion in lupus nephritis (LN), is rare but can be life-threatening. Early recognition and prompt treatment have significant impacts on clinical outcomes. A 28-year-old female with clinical manifestations of thrombocytopenia, severe anemia, and renal failure was diagnosed with systemic lupus erythematosus. However, the platelet count declined continuously, and hemolysis persisted even after pulse infusion of glucocorticoid. A disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity test (50.3%) and kidney biopsies confirmed the diagnosis of LN combined with TMA. After timely plasmapheresis, the serum creatinine level returned to normal and stabilized over the follow-up of six years. Patients with LN presenting with a continuous decline in platelet count, even after intensive glucocorticoid and immunosuppressant therapy, should pay attention to the potential occurrence of TMA. Timely ADAMTS13 activity testing and kidney biopsies assist in early diagnosis, and plasmapheresis may improve patient outcomes.