Adult-onset still’s disease with macrophage-activation syndrome presentation in a critically ill woman in the second trimester of pregnancy: A case report

Abstract

Background: A rare inflammatory illness known as Adult-Onset Still’s Disease (AOSD) is typically characterized by a high fever, arthralgias, and a transient rash of salmon hue. Systemic immune hyperactivation syndrome known as Hemophagocytic Lymphohistiocytosis (HLH) can occur as a primary process in those with a genetic predisposition or it can be brought on by another disease, frequently a viral infection or cancer. When a rheumatologic condition serves as the trigger, secondary HLH is referred to as Macrophage Activation Syndrome (MAS). Reduced natural killer (NK) cell activity, increased soluble interleukin-2 receptor (sIL-2R), and generally, excessive cytokine production appear to be components of both AOSD and MAS pathogenesis.