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Macrophage activation syndrome is a severe, potentially life-threatening, complication of several chronic rheumatic diseases of childhood. It occurs most commonly with systemic-onset juvenile idiopathic arthritis (SoJIA).
In rheumatic diseases, this syndrome is more often referred to as macrophage activation syndrome (MAS) and occurs most frequently in the juvenile onset and adult onset forms of Still's disease and in systemic lupus erythematosus. It occurs rarely in juvenile idiopathic arthritis, juvenile Kawasaki disease, and rheumatoid arthritis. [7]
Excessive activation of neutrophils, monocytes/macrophages and dendritic cells leads to auto-inflammatory symptoms, while T cell and B cell dysfunction leads to autoimmunity. Failure of innate and/or adaptive immune cells to appropriately activate, recognize, and clear infectious agents causes immunodeficiency and vulnerability to infection.
Macrophage activation syndrome. [3] Usual onset: 1-5 years old. [2] Diagnostic method: Excluding other disorders and clinical criteria. [2] Differential diagnosis: Septic arthritis, osteomyelitis, postinfectious arthritis, multisystem inflammatory syndrome in children, malignancy, and other autoimmune and autoinflammatory diseases. [2] Treatment
Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication that can occur in patients with the systemic subtype of JIA. MAS involves uncontrolled activation of the immune system, sometimes referred to as a 'cytokine storm', which can present with a sepsis -like picture of fever, rash, enlarged liver and spleen ...
Cytokine release syndrome (CRS) Hemophagocytic lymphohistiocytosis, macrophage activation syndrome, and CRS are common side effects after CAR-T treatment. [8] Main articles: Cytokine release syndrome; chimeric antigen receptor T cell. IgE-mediated anaphylactic reaction
Cytokine storm syndrome is a diverse set of conditions that can result in a cytokine storm. Cytokine storm syndromes include familial hemophagocytic lymphohistiocytosis , Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis, systemic or non-systemic juvenile idiopathic arthritis –associated macrophage activation syndrome , NLRC4 ...
A macrophage-activating factor (MAF) is a lymphokine or other receptor based signal that primes macrophages towards cytotoxicity to tumors, cytokine secretion, or clearance of pathogens. Similar molecules may cause development of an inhibitory, regulatory phenotype.