Macrophage activation syndrome in a patient with systemic lupus erythematosus (SLE) and dual viremia

Abstract

Macrophage Activation Syndrome (MAS) is a potentially fatal inflammatory condition that can rapidly lead to multi-organ failure if inadequately treated. Also, known as secondary Hemophagocytic Lymphohistiocytosis (sHLH), MAS is commonly seen as a complication of systemic inflammatory disorders, like systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE). However, MAS can also present as a complication of malignancies and infections, particularly viral infections like Epstein-Barr virus (EBV), cytomegalovirus (CMV) and HIV. Here we describe a patient with an underlying history of SLE and Sjogren's disease who was found have both EBV and CMV infections, presented to our facility with fever, lymphadenopathy, pneumonia and pancytopenia. Patient was treated in line with sepsis in the intensive care unit but rapidly developed multigrain failure despite early aggressive treatment. As will be discussed below, patient had characteristic signs and symptoms of MAS with biochemical parameters pointing towards the same.