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Macrophage Activation Syndrome
1Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Abstract:
Macrophage activation syndrome (MAS) is a state of immune hyperactivation that can result in life-threatening multisystem end-organ dysfunction. Often termed a "cytokine storm," MAS occurs among the rheumatic diseases most typically in Still's disease but also in systemic lupus erythematosus and Kawasaki disease. MAS can also accompany infection, malignancy, and inborn errors of immunity. This review provides a practical, evidence-based guide to the understanding, recognition, and management of MAS in children and adults, with a primary focus on MAS complicating Still's disease.
Insights
Macrophage activation syndrome (MAS) is a severe immune overreaction, often called a cytokine storm, that can damage multiple organs. This review guides understanding, recognizing, and managing MAS, especially in Still's disease.
Area of Science:
- Rheumatology
- Immunology
- Pediatrics
Background:
- Macrophage activation syndrome (MAS) is a critical condition characterized by immune hyperactivation.
- It can lead to severe, life-threatening multisystem end-organ dysfunction, often referred to as a "cytokine storm."
- MAS is frequently associated with rheumatic diseases like Still's disease, systemic lupus erythematosus, and Kawasaki disease, but can also occur with infections, malignancies, and primary immune deficiencies.
Purpose of the Study:
- To provide a practical, evidence-based guide for the recognition and management of MAS.
- To focus primarily on MAS complicating Still's disease in both pediatric and adult populations.
- To enhance the clinical understanding and timely intervention for MAS.
Main Methods:
- This review synthesizes current evidence and clinical guidelines.
- It focuses on practical aspects of diagnosis and treatment.
- Expert consensus and literature review form the basis of recommendations.
Main Results:
- MAS presents a diagnostic challenge due to overlapping symptoms with underlying conditions.
- Early recognition and prompt, aggressive treatment are crucial for improving outcomes.
- Management strategies involve immunosuppression and supportive care, tailored to the specific clinical context.
Conclusions:
- MAS is a serious, potentially fatal complication requiring prompt recognition and management.
- A systematic approach to diagnosis and treatment, particularly in Still's disease, can improve patient survival and reduce morbidity.
- Further research is needed to refine diagnostic criteria and optimize therapeutic strategies for MAS across various etiologies.
