Questioning the source of
nonspecific hyperinflammation?
It could be HLH/MAS
Macrophage activation syndrome (MAS) is a rare and potentially fatal syndrome that can occur in patients with underlying rheumatic disease. MAS is characterized by interferon gamma (IFNγ)-hyperactivated macrophages that release an uncontrolled surge of proinflammatory cytokines leading to life-threatening hyperinflammation.1-3
Find out how to identify this syndrome of dangerous immune dysregulation in your patients before it’s too late.
MULTISYSTEM DISTRESS4-7
MULTISYSTEM DISTRESS4-7
Signs of general malaise
Persistent high fever
Central nervous system involvement
Rash
Hyperferritinemia
Hepatosplenomegaly
Hypertriglyceridemia
Coagulopathies
This is not an exhaustive list of MAS signs and symptoms. Presentation may vary between patients, and it is not necessary to identify all of these symptoms to diagnose MAS.
Macrophage activation syndrome (MAS) is a rare and potentially fatal syndrome that can occur in patients with underlying rheumatic disease. MAS is characterized by interferon gamma (IFNγ)-hyperactivated macrophages that release an uncontrolled surge of proinflammatory cytokines leading to life-threatening hyperinflammation.1-3
Find out how to identify this syndrome of dangerous immune dysregulation in your patients before it’s too late.
References: 1. Schulert GS, Grom AA. Macrophage activation syndrome and cytokine-directed therapies. Best Pract Res Clin Rheumatol. 2014;28(2):277-292. doi:10.1016/j.berh.2014.03.002 2. Crayne C, Cron RQ. Pediatric macrophage activation syndrome, recognizing the tip of the iceberg. Eur J Rheumatol. 2020;7(Suppl1):S13-S20. doi:10.5152/eurjrheum.2019.19150 3. Di Cola I, Ruscitti P, Giacomelli R, Cipriani P. The pathogenic role of interferons in the hyperinflammatory response on adult-onset Still’s disease and macrophage activation syndrome: paving the way towards new therapeutic targets. J Clin Med. 2021;10(6):1164. doi:10.3390/jcm10061164 4. Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol. 2018;10:117-128. doi:10.2147/OARRR.S151013 5. Sen ES, Clarke SL, Ramanan AV. Macrophage activation syndrome. Indian J Pediatr. 2016;83(3):248-253. doi:10.1007/s12098-015-1877-1 6. Monteagudo LA, Boothby A, Gartner E. Continuous intravenous anakinra infusion to calm the cytokine storm in macrophage activation syndrome. ACR Open Rheumatol. 2020;2(5):276-282. doi:10.1002/acr2.11135 7. Avau A, Matthys P. Therapeutic potential of interferon-γ and its antagonists in autoinflammation: lessons from murine models of systemic juvenile idiopathic arthritis and macrophage activation syndrome. Pharmaceuticals (Basel). 2015;8(4):793-815. doi:10.3390/ph8040793
