title: [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].
abstract: Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.
authors.affiliation: Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil. Electronic address: annafmgomes@hotmail.com.
authors.forename: Anna Carolina Faria Moreira Gomes
authors.initials: AC
authors.lastname: Tavares
authors.affiliation: Departamento do Aparelho Locomotor, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
authors.forename: Gilda Aparecida
authors.initials: GA
authors.lastname: Ferreira
authors.affiliation: Serviço de Reumatologia, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
authors.forename: Luciano Junqueira
authors.initials: LJ
authors.lastname: Guimarães
authors.affiliation: Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
authors.forename: Raquel Rosa
authors.initials: RR
authors.lastname: Guimarães
authors.affiliation: Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
authors.forename: Flávia Patrícia Sena Teixeira
authors.initials: FP
authors.lastname: Santos
mesh_terms: D001171:Arthritis, Juvenile / Q000150:complications*; D002648:Child; D005260:Female; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000209:etiology*
keywords: Artrite idiopática juvenil forma sistêmica; Epstein‐Barr virus; HLH‐04 Treatment protocol; Hemophagocytic lymphohistiocytosis; Protocolo de tratamento HLH‐04; Systemic‐onset juvenile idiopathic arthritis; Síndrome hematofagocítica; Vírus Epstein‐Barr