title: Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood.
abstract: 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.
authors.affiliation: Division of Rheumatology and Department of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Tadej
authors.initials: T
authors.lastname: Avcin
authors.forename: Shirley M L
authors.initials: SM
authors.lastname: Tse
authors.forename: Rayfel
authors.initials: R
authors.lastname: Schneider
authors.forename: Bo
authors.initials: B
authors.lastname: Ngan
authors.forename: Earl D
authors.initials: ED
authors.lastname: Silverman
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001171:Arthritis, Juvenile / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D005260:Female; D006801:Humans; D007223:Infant; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D008262:Macrophage Activation; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D011956:Receptors, Cell Surface / Q000378:metabolism; D013577:Syndrome; D000098902:CD163 Antigen
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D000098902:CD163 Antigen; D011956:Receptors, Cell Surface