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Vol 23. no 1
March 2018

Macrophage activation syndrome (MAS) associated with connective tissue diseases (summary)
Joanna Zalewska, Katarzyna Napiórkowska-Baran, Krzysztof Pałgan, Sławomir Jeka1, Zbigniew Bartuzi
Macrophage activation syndrome (MAS) is a reactive form of haemophagocytic lymphohistiocytosis (HLH). The mechanisms causing the disease include activation and proliferation of macrophages, which is associated with symptoms of systemic inflammatory reaction, enlargement of organs and cytopenia due to hemophagocytosis of hematopoietic cells. MAS may develop in the course of systemic connective tissue diseases, most commonly in juvenile idiopathic arthritis, Still’s disease and systemic lupus erythematosus. In clinical image is characterized by persistent fever despite the use of antibiotics, hepatosplenomegaly, lymph node enlargement, rash, neurological symptoms, hepatic and myocardial insufficiency are observed. It is a serious, life-threatening and potentially fatal complication. Therefore, proper diagnostic process and providing optimal treatment is crucial. In the diagnostic process the HScore scale can be used to assess the probability of MAS occurrence in a patient. In the case of MAS secondary to systemic connective tissue diseases, the treatment should be considered individually depending on the underlying disease, triggering factor and the severity of symptoms. The first line of treatment are intravenous glucocorticosteroids. In case of ineffective treatment, cyclosporin A, intravenous immunoglobulin, etoposide, plasmapheresis, anti-thymocyte globulin, granulocyte-macrophage colony-stimulating factor (GM-CSF) as well as biological treatment are used.
Keywords: macrophage activation syndrome, connective tissue diseases, juvenile idiopathic arthritis, Still’s disease
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