Macrophage Activation Syndrome in Adults: A Retrospective Case Series

Abstract

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

Macrophage Activation Syndrome Originating from Primary Biliary Cholangitis: A Case Report

“…Macrophage activation syndrome (MAS), also known as secondary or reactive hemophagocytic Lymphohistiocytosis (HLH), is a clinical syndrome caused by excessive immune activation leading to cytokine storm in the body [1]. Previous MAS is noted in pediatric patients with systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematous (SLE) [2]. The MAS in adult rheumatic diseases most commonly develops with AOSD and SLE [3].…”

Atypical Presentation of Macrophage Activation Syndrome Associated with Primary Sjogren\'s Syndrome

“…4 The pathophysiology of MAS is unclear. 5 Presently, it is thought that MAS is due to a combination of genetic mutations affecting the functionality of cytolysis. The absence of physiologic cytolysis prevents natural killer cell and cytotoxic T-cell mediated suppression of activated lymphocytes and results in a cytokine storm, most notably mediated by Interferon-γ and interleukin-18 (Fig.…”