Makay Balahan, Yilmaz Sebnem, Türkyilmaz Zeynep, Unal Nurettin, Oren Hale, Unsal Erbil
Department of Pediatric Rheumatology, Dokuz Eylul University Faculty of Medicine, Balcova, Izmir, Turkey.
Pediatr Blood Cancer. 2008 Feb;50(2):419-21. doi: 10.1002/pbc.21019.
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.
巨噬细胞活化综合征（MAS）是儿童风湿性疾病的一种严重且可能致命的并发症，尤其是全身型幼年特发性关节炎（SoJIA）。我们报告了一名4岁可能患有SoJIA的女孩，她出现了MAS。她对静脉注射甲泼尼龙和环孢素A（CsA）无反应。她对静脉注射免疫球蛋白（IVIG）治疗也无反应。基于对MAS患者血清肿瘤坏死因子-α（TNF-α）水平升高的观察，开始使用依那西普治疗。使用依那西普后她的病情有所改善，这表明依那西普可能对难治性MAS具有治疗作用。
