Etanercept for therapy-resistant macrophage activation syndrome.
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.
Makay
Balahan
B
Department of Pediatric Rheumatology, Dokuz Eylul University Faculty of Medicine, Balcova, Izmir, Turkey. balahan.bora@deu.edu.tr
Yilmaz
Sebnem
S
Türkyilmaz
Zeynep
Z
Unal
Nurettin
N
Oren
Hale
H
Unsal
Erbil
E
D002675:Child, Preschool; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy* / Q000276:immunology; D008262:Macrophage Activation; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*
D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D000068800:Etanercept