Combination Therapy of Plasma Exchange and Intravenous Cyclophosphamide in a Case with Thrombotic Microangiopathy and Systemic Lupus Erythematosus: A Case Report and Literature Review

Abstract

A 17-year-old girl was admitted due to systemic lupus erythematosus (SLE) with disease flare with arthritis, renal dysfunction and abnormal hemogram. The patient responded poorly to high-dose steroid therapy. Although the patient did not have neurological symptoms or fever, laboratory tests identified microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Therefore, SLE with disease flare complicated by thrombotic microangiopathy (TMA) was diagnosed. The patient later responded to therapy with aggressive plasma exchange and cytotoxic agents. TMA is a rare hematological life-threatening disorder that can coexist with SLE. Manifestations of TMA are similar to those in SLE and, consequently, TMA can be misdiagnosed as SLE flare. Additionally, TMA has good response to plasma exchange and SLE is typically well controlled with steroids or cytotoxic agents. There is no documented therapy for patients with SLE and TMA. Therefore this report describes a lupus patient with disease flare combined with TMA who was successfully treated with combination therapy of plasma exchange and intravenous cyclophosphamide.