Revista chilena de pediatría
versión impresa ISSN 0370-4106
GONZALEZ M., Benito; ROA A., Johanna y SCHMIDT S., Nadia. Activated macrophage syndrome in paediatrics: 4 clinical cases. Rev. chil. pediatr. [online]. 2005, vol.76, n.2, pp. 183-192. ISSN 0370-4106. http://dx.doi.org/10.4067/S0370-41062005000200010.
Introduction: The macrophage activation syndrome (MAS) is a rare paediatric condition characterized by enhanced activation of the macrophage and T-cell system, with increased liberation of T-cell cytokines. Clinically it resembles a multiorgan failure syndrome of infectious aetiology. There is still some debate about the syndromes nomenclature, hemato-oncologists usually classify this syndrome as a histiocytic disorder, while rheumatologists define it as MAS. The objective of this publication is to describe 4 patients in which the diagnosis of MAS was made in the early stages of the disease. Two patients had juvenile idiopathic artritis (systemic presentation), 1 with probable immunodeficiency, and in the last case, a familial histiocytosis was suspected. Trigger factors were drugs in 2 patients and 2 infections in the remaining cases. Conclusions: The relevance of this report is to show that the early diagnosis and aggressive treatment with steroids and cyclosporine can improve the prognosis of this rare syndrome
Palabras llave : MAS; juvenile idiopathic artritis; systemic juvenile idiopathic artritis; immunodeficienc; histiocytosis.