Etanercept for therapy-resistant macrophage activation syndrome

Makay B., YILMAZ Ş., Tuerkyilmaz Z., Uenal N., ÖREN H., Uensal E.

PEDIATRIC BLOOD & CANCER, no.2, pp.419-421, 2008 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Publication Date: 2008
  • Doi Number: 10.1002/pbc.21019
  • Journal Name: PEDIATRIC BLOOD & CANCER
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.419-421
  • Keywords: etanercept, macrophage activation syndrome, TNF-alpha inhibition, HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS, RHEUMATOID-ARTHRITIS, ABNORMALITIES, DISEASE
  • Dokuz Eylül University Affiliated: Yes

Abstract

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of childhood rheumatic diseases, especially systemic onset juvenile idiopathic arthritis (SoJIA). We report a 4-year-old girl with probable SoJIA who presented with MAS. She did not respond to pulse methyl prednisolone and Cyclosporine A (CsA). She also failed to respond to intravenous immunoglobulin (IVIG) therapy. Etanercept was started, based on the observation of increased serum levels of tumor necrosis factor-alpha (TNF-alpha) in patients with MAS. Her condition improved following etanercept, suggesting that etanercept might have a therapeutic role in resistant MAS.