An Unusual Case of Macrophage Activation Syndrome (MAS)- Hemophagocytic Lymphohistiocytosis (HLH) Triggered by Necrotizing Autoimmune Myopathy

Tyiesha S Brown; Gregory Vo; Prangthip Charoenpong

doi:10.7759/cureus.38501

An Unusual Case of Macrophage Activation Syndrome (MAS)- Hemophagocytic Lymphohistiocytosis (HLH) Triggered by Necrotizing Autoimmune Myopathy

Cureus. 2023 May 3;15(5):e38501. doi: 10.7759/cureus.38501. eCollection 2023 May.

Authors

Tyiesha S Brown¹, Gregory Vo², Prangthip Charoenpong³

Affiliations

¹ Internal Medicine, Louisiana State University Health Sciences Center, Shreveport, USA.
² Critical Care Medicine, Louisiana State University Health Sciences Center, Shreveport, USA.
³ Pulmonology and Critical Care, Louisiana State University Health Sciences Center, Shreveport, USA.

Abstract

Macrophage activation syndrome (MAS)- hemophagocytic lymphohistiocytosis (HLH) secondary to inflammatory myopathies such as dermatomyositis (DM), polymyositis (PM), and necrotizing autoimmune myopathy is exceedingly rare in the medical literature. We present the complicated diagnosis and treatment of a 41-year-old female who presented with proximal muscle weakness and shock. Following an extensive critical care workup, she was diagnosed with MAS-HLH, triggered by a newly diagnosed necrotizing autoimmune myopathy. In this case report and literature review, we would like to highlight the importance of recognizing the clinical signs of MAS-HLH in rheumatological disorders and the necessity for rapid treatment.

Keywords: acute polymyositis; critical care; hemophagocytic lymphohistiocytosis (hlh); macrophage activation syndrome (mas); medical critical care; s: dermatomyositis.

Publication types

Case Reports