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J Pediatr. 2006 May;148(5):683-6.

Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood.

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  • 1Division of Rheumatology and Department of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.


We describe 3 patients who presented with features of macrophage activation syndrome (MAS) at the time of presentation of systemic lupus erythematosus (SLE), systemic juvenile idiopathic arthritis, and Kawasaki disease. Immunohistochemical studies in the patient with SLE demonstrated extensive expression of CD163 on hemophagocytic macrophages, suggesting a possible role as a marker of MAS.

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