Macrophage Activation Syndrome

Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

Authors: Halyabar O, Chang MH, Schoettler ML, Schwartz MA, Baris EH, Benson LA, Biggs CM, Gorman M, Lehmann L, Lo MS, Nigrovic PA, Platt CD, Priebe GP, Rowe J, Sundel RP, Surana NK, Weinacht KG, Mann A, Yuen JC, Meleedy-Rey P, Starmer A, Banerjee T, Dedeoglu F, Degar BA, Hazen MM, Henderson LA

Summary: A multidisciplinary group of physicians at Boston Children’s developed a novel evidence-based guideline (EBG) for the treatment of hemophagoytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), two closely related inflammatory diseases. The goal of this EBG is to bolster communication between physicians to shorten the time it takes to diagnose patients and standardize treatment to ultimately improve patient outcomes.

Publication Information: Pediatric Rheumatology, February 2019

Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Authors: Lee PY, Schulert GS, Canna SW, Huang Y, Sundel J, Li Y, Hoyt KJ, Blaustein RB, Wactor A, Do T, Halyabar O, Chang MH, Dedeoglu F, Case SM, Meidan E, Lo MS, Sundel RP, Richardson ET, Newburger JW, Hershfield MS, Son MB, Henderson LA, Nigrovic PA

Summary: In this study the authors used samples from patients to study the utility of using ADA2, a protein, as a biomarker of macrophage activation syndrome. A specific score related to this protein was identified as a reliable and novel biomarker of the disease.

Publication Information: Annals of the Rheumatic Diseases, November 2019