Articles and Guidelines

• EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease
• Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
  
• Macrophage Activation Syndrome (Review by Peter A. Nigrovic - 2025)
  
• Macrophage Activation Syndrome: Not Just for Rheumatologists Anymore (book chapter 2025)
  
• Ruxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome​ (2025)
  
• Targeting JAK-STAT in macrophage activation syndrome—no MAS! (comment) 2025
  

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• The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS)  PDF file
• Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome PDF file. Pediatr Rheumatol Online J. 2019 Feb 14;17(1):7.
• Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome PDF file. Lancet Rheumatol 2020; 2: 358–67
• An Evidence-Based Guideline Improves Outcomes for Patients With Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome PDF file. The Journal of Rheumatology 2022;49:1042–51
• Emapalumab Treatment in Patients With Rheumatologic Disease–Associated HLH in the USA: A Retrospective Medical Chart Review Study. Arthritis & Rheumatology. 2025;77(2):226-238
• Recent advances and evolving concepts in Still’s disease (Nature Review 2024). PDF file. Nat Rev Rheumatol 20, 116–132 (2024)
• Does your unwell patient have haemophagocytic lymphohistiocytosis? PDF file. Br J Hosp Med (Lond). 2024 Mar 2;85(3):1-12.
• Heterogeneity of macrophage activation syndrome and treatment progression​. PDF file. Front Immunol. 2024 Apr 26;15:1389710.
• Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin. PDF file. Sztajnbok et al. Advances in Rheumatology (2024)064:280

Online Resources:

• H-Score: MDCalc Online Calculator