Systemic Lupus Erythematosus With Hemophagocytic Lymphohistiocytosis: Is COVID-19 the Inciting Factor?

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that is characterized by an aberrant immune response leading to immune-mediated damage to tissues. Hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition, consists of a constellation of symptoms caused by excessive immune activation and cytokine storm. HLH is categorized into the primary and secondary form. The secondary form is often referred to as the macrophage activation syndrome. HLH in the background of SLE is a rare and potentially fatal entity. It is often seen in the context of disease flare and is rarely associated with the initial diagnosis of SLE. Severe infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes a cytokine storm characterized by marked elevation of inflammatory markers including ferritin. Here, we describe the case of a young female with an inaugural diagnosis of SLE and features of HLH after a recent SARS-CoV-2 infection.