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[Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis]

Anna Carolina Faria Moreira Gomes Tavares1, Gilda Aparecida Ferreira2, Luciano Junqueira Guimarães3

  • 1Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.

Revista Brasileira De Reumatologia
|January 11, 2015
PubMed

Insights

Macrophage activation syndrome (MAS) is a severe condition often linked to juvenile idiopathic arthritis. This case highlights successful treatment of EBV-associated MAS in a patient with systemic juvenile idiopathic arthritis using the 2004-HLH protocol.

Area of Science:

  • Rheumatology
  • Immunology
  • Pediatrics

Background:

  • Macrophage activation syndrome (MAS) is a rare, life-threatening hyperinflammatory condition.
  • It is frequently associated with chronic rheumatic diseases, particularly juvenile idiopathic arthritis (JIA).
  • MAS is a secondary form of hemophagocytic lymphohistiocytosis (HLH), with infections and lymphoproliferative diseases as other causes.

Observation:

  • Key clinical features include persistent fever, splenomegaly, bleeding, liver dysfunction, cytopenias, and characteristic laboratory abnormalities (hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia).
  • Epstein-Barr virus (EBV) is a recognized potential trigger for MAS, especially in patients on TNF blockers.
  • Refractory cases may require etoposide (VP16) in addition to corticosteroids and cyclosporine.

Findings:

  • This report details a rare case of MAS likely triggered by EBV infection in a patient with systemic-onset JIA.
  • The patient achieved complete disease remission following treatment guided by the 2004-HLH protocol.
  • This therapeutic approach underscores the efficacy of established HLH protocols for complex MAS cases.

Implications:

  • The findings suggest that the 2004-HLH protocol can be effective in managing EBV-associated MAS in patients with systemic JIA.
  • Early recognition and prompt treatment are crucial for favorable outcomes in MAS.
  • This case contributes to understanding MAS pathogenesis and treatment strategies, particularly in the context of rheumatic diseases and viral triggers.
Keywords:
Artrite idiopática juvenil forma sistêmicaEpstein‐Barr virusHLH‐04 Treatment protocolHemophagocytic lymphohistiocytosisProtocolo de tratamento HLH‐04Systemic‐onset juvenile idiopathic arthritisSíndrome hematofagocíticaVírus Epstein‐Barr

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