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
- Macrophage Activation Syndrome - Review by Peter A. Nigrovic. Arthritis Rheumatol . 2025 Apr;77(4):367-379. doi: 10.1002/art.43052. Epub 2024 Dec 12.
- 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:
Educational Materials
- RheumGuide.ca MAS/HLA infographic (version May 2026)
- Adult MAS/HLH: Early Recognition Trigger Guide (Draft May 2026)
- Video Presentation (Draft)
- MAS/HLH Standardized Lab Order Set example (Draft)
- MAS/HLH Specialized Testing: Practical Guidance on When to Order, When Not to Order, and Why (Draft)
- MAS/HLH Case Learning Template (Draft)
