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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).
This shows that even single-cytokine dysregulation can cause autoinflammatory diseases. Some mutations can change the ability of cytotoxic cells to induce cell death, failing to terminate macrophage and dendritic cell activation and causing macrophage activation syndrome. [2]
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]
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
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
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 ...
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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.