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Background: Macrophagic activation syndrome (MAS) may appear as a complication in patients with rheumatic diseases, it has been more frequently associated with the systemic form of juvenile idiopathic arthritis and systemic lupus erythematosus.
Case report: We present the case of a school-aged patient, who debuted with MAS as a complication of juvenile systemic idiopathic arthritis, refractory to conventional treatments and with an adequate response to biological therapy.
Discussion: MAS is a life-threatening autoinflammatory disorder, characterized by the activation of the intracellular inflamosoma with proliferation of T lymphocytes and the mononuclear phagocytic system, and hyperproduction of inflammatory prostaglandins such as IL 1, IL 6 and IL 18; its diagnosis is based on a combination of clinical signs and biochemical and histological abnormalities.
Palabras clave: Macrophage activation syndrome, inflammation, arthritis juvenile.