Macrophage Activation Syndrome in Adults: A Retrospective Case Series. (June 2021)

• Record Type: Journal Article
• Authors: Warmoth, Taylor; Ramesh, Malvika; Iwuji, Kenneth; Pixley, John S.
• Is Part Of: Journal of investigative medicine high impact case reports. Volume 9(2021)
• Publication Date: 2021-06
• Subjects: macrophage activation syndrome -- adults; Internal medicine -- Periodicals; Medical care -- Periodicals; 616.005
• DOI: 10.1177/23247096211026406
• Languages: English
• ISSNs: 2324-7096

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.