Macrophage Activation Syndrome complicated with acute pancreatitis as the first presentation of Systemic lupus Erythematosus

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly acquired macrophages and cytotoxic T cells. The primary form is called the Genetic form where as the secondary acquired form is most common in adults. Secondary HLH induced by autoinflammatory or autoimmune disorders is called Macrophage activation syndrome (MAS or MAS-HLH). Here we present a case of Systemic lupus Erythematosus presenting for the first time with MAS-HLH associated with acute pancreatitis, emphasizing the challenges in early detection and the need for prompt treatment, given the high mortality associated with MAS-HLH.