title: Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide.
abstract: The macrophage activation syndrome is a rare but potentially fatal complication of patients with autoimmune rheumatic diseases. This is a clinicopathological entity characterized by activation of histiocytes with prominent hemophagocytosis in the bone marrow and other reticuloendothelial systems. In patients with lupus it may mimic an exacerbation of the disease or infection. We report the case of a 7-year-old girl in whom the diagnosis of lupus erythematosus and macrophage activation syndrome was simultaneously made with response to the use of cyclophosphamide.
authors.affiliation: Reumatología Pediátrica, Hospital General Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México D.F., México. Electronic address: dr-poncho@hotmail.com.
authors.forename: Alfonso
authors.initials: A
authors.lastname: Torres Jiménez
authors.affiliation: Reumatología Pediátrica, Hospital General Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México D.F., México.
authors.forename: Eunice
authors.initials: E
authors.lastname: Solís Vallejo
authors.affiliation: Reumatología Pediátrica, Hospital General Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México D.F., México.
authors.forename: Maritza
authors.initials: M
authors.lastname: Zeferino Cruz
authors.affiliation: Reumatología Pediátrica, Hospital General Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México D.F., México.
authors.forename: Adriana
authors.initials: A
authors.lastname: Céspedes Cruz
authors.affiliation: Hematología Pediátrica, Hospital General Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México D.F., México.
authors.forename: Berenice
authors.initials: B
authors.lastname: Sánchez Jara
mesh_terms: D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D055501:Macrophage Activation Syndrome / Q000188:drug therapy* / Q000209:etiology*; D012074:Remission Induction; D012720:Severity of Illness Index
chemical_list: D003520:Cyclophosphamide
keywords: Ciclofosfamida; Cyclophosphamide; Juvenile systemic lupus erythematosus; Lupus eritematoso sistémico juvenil; Macrophage activation syndrome; Síndrome de activación de macrófago