Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura

Abstract

Thrombotic Thrombocytopenic Purpura (TTP) is a rare form of thrombotic microangiopathy (TMA) typically characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and end-organ ischemia secondary to microvascular dissemination of platelet-rich thrombi. Systemic Lupus Erythematosus (SLE) is a chronic autoimmune condition characterized by multi-organ system inflammation with the generation of autoantibodies. The specific cause of SLE is unknown but multiple factors seem to be associated with the development of the disease including: genetic, epigenetic, ethnic, hormonal, and environmental factors. In SLE, patients may present with a wide assortment of clinical variations, but diagnosis is based generally on clinical and laboratory findings after excluding alternative diagnoses. Very rarely do SLE and TTP present simultaneously. Both disease processes have overlapping clinical features which makes diagnosis and management challenging. The following case report describes a forty-year-old female with no prior history of SLE or TTP who presented with clinical findings and features of TTP as the initial clinical manifestation of her underlying SLE, as well as serologic criteria, requiring treatment with plasma exchange, high dose steroids, and eventual outpatient infusion with Rituximab.