Macrophage Activation Syndrome (MAS) is a life-threatening hyperinflammatory condition (cytokine storm) occurring as a severe complication of rheumatic diseases such as Still's disease, systemic lupus erythematosus, and Kawasaki disease.

The findings of the international research project METAPHOR have been published in "Arthritis & Rheumatology", evaluating real-world clinical practice and establishing future priorities.

Key Findings and Conclusions

  1. Treatment Heterogeneity: Management strategies for MAS vary significantly globally depending on physician specialty, regional protocols, and drug availability.
  2. First-Line Therapy: Glucocorticoids (methylprednisolone) remain the cornerstone of treatment, though early initiation of targeted immunomodulators is recommended to reduce steroid toxicity.
  3. Biologic and Targeted Therapies: Anakinra (IL-1 inhibitor) and Emapalumab (IFNγ inhibitor) showed high efficacy in MAS associated with Still's disease. Ruxolitinib (JAK inhibitor), Cyclosporine A, and IVIG are also widely used.

Future Priorities

  • Ensuring global equitable access to advanced targeted therapies in underserved regions.
  • Developing standardized clinical guidelines for MAS associated with various underlying autoimmune conditions.