Etanercept for therapy-resistant macrophage activation syndrome.

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.

• Authors: Balahan Makay, Sebnem Yilmaz, Zeynep Türkyilmaz, Nurettin Unal, Hale Oren, Erbil Unsal
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 50 Issue 2 Pg. 419-21 (Feb 2008) ISSN: 1545-5017 [Electronic] United States
• PMID: 16900487 (Publication Type: Journal Article)
• Copyright: (c) 2007 Wiley-Liss, Inc.

Chemical References

• Immunoglobulin G
• Receptors, Tumor Necrosis Factor
• Etanercept

Topics

• Child, Preschool
• Etanercept
• Female
• Humans
• Immunoglobulin G (therapeutic use)
• Lymphohistiocytosis, Hemophagocytic (drug therapy, immunology)
• Macrophage Activation
• Receptors, Tumor Necrosis Factor (therapeutic use)