Macrophage activation syndrome in a pediatric patient suffering from Still's disease

Abstract

Introduction: Still's disease in childhood constitutes an inflammatory disorder, of autoimmune origin, which is framed within the group of juvenile idiopathic arthritis, probably being the most peculiar entity of the group. It usually occurs in the form of repeated outbreaks of activity, combined with periods of remission.
Case report: a sixteen-year-old patient with a history of systemic juvenile arthritis is presented, with a clinical picture of 72 hours of evolution characterized by intermittent fever, cough, pyodermitis, generalized rash and arthritis. During the clinical evolution, it was considered base-disease activation versus sepsis supported on clinical criteria, which was later corroborated by laboratory findings. The patient underwent treatment with broad-spectrum antibiotics, non-steroidal anti-inflammatory drugs, steroids at a rate of 1mg / kg / day, without improvement. Subsequently, the treatment with pulses of methylprednisolone and immunomodulator-therapy was initiated. Finally, a macrophage activation syndrome was diagnosed, supported on clinical laboratory parameters. Patient’s evolvement was slowly and died in the Intensive Care Unit, 22 days after the admission.
Conclusions: the macrophage activation syndrome is one of the secondary forms within the group of hemophagocytic lymphohistiocytosis. The factors that in this context have been associated with its onset are very diverse. Clinically, these processes are characterized by prolonged fever that does not disappear despite antibiotic-therapy. Typically, arthritis is usually absent. The first line of treatment is corticosteroids at high doses.