Abstract

STERBA, Gary; STERBA, Yonit  and  IGLESIAS G, Antonio. Macrophage activation syndrome in adults with rheumatic disease. Rev.Colomb.Reumatol. [online]. 2016, vol.23, n.2, pp.137-143. ISSN 0121-8123.

Introduction: Macrophage activation syndrome (MAS) is a pathological systemic inflammatory reaction that is often fatal and underdiagnosed. There may be multiple organ failure that could be triggered in association with rheumatic, neoplastic or infectious diseases and/or drugs. It has been reported more in children than adults, probably as it is often associated with genetic abnormalities not described yet undescribed, genetic abnormalities. In most cases the genetic defect is not recognized in adults, or has a different etiology. The signs and symptoms of macrophage activation syndrome have been defined. Not suspecting its presence may lead to not making the diagnosis and thus, an increase in mortality. Diagnosis is a challenge, treatment has to be started early and be aggressive to reduce the high mortality rate. Objectives: To describe four adult patients with five MAS episodes related to different under-lying diseases, with the aim of making it familiar to the reader, to look for the syndrome and make a diagnosis. Materials and methods: Patients evaluated in outpatients and while in the hospital. Results: We present the characteristics of MAS, with the diagnostic approach and the ther-apeutic possibilities and their outcomes. Conclusions: MAS is not looked for in the adult and could be fatal. It requires identification and early treatment to reduce the risk of mortality. It still needs to be studied to define the genetic defect, or other causes that may be responsible for the development of the syndrome.

Keywords : Macrophage activation syndrome; Reactive lymphohistiocytic; haemophagocytosis.

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