• Reumatología clinica · Sep 2014

    Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide.

    • Alfonso Torres Jiménez, Eunice Solís Vallejo, Maritza Zeferino Cruz, Adriana Céspedes Cruz, and Berenice Sánchez Jara.
    • Reumatología Pediátrica, Hospital General Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, México D.F., México. Electronic address: dr-poncho@hotmail.com.
    • Reumatol Clin. 2014 Sep 1;10(5):331-5.

    AbstractThe 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.Copyright © 2013 Elsevier España, S.L. All rights reserved.

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