Etanercept for therapy‐resistant macrophage activation syndrome
- 9 August 2006
- journal article
- research article
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 50 (2), 419-421
- https://doi.org/10.1002/pbc.21019
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-α) in patients with MAS. Her condition improved following etanercept, suggesting that etanercept might have a therapeutic role in resistant MAS. Pediatr Blood Cancer 2008;50:419–421.Keywords
This publication has 13 references indexed in Scilit:
- Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?Current Opinion in Rheumatology, 2003
- Macrophage Activation Syndrome in a Patient with Systemic Onset Rheumatoid ArthritisJCR: Journal of Clinical Rheumatology, 2003
- Haemophagocytic syndrome in a rheumatoid arthritis patient treated with infliximabRheumatology, 2003
- Macrophage activation syndrome: a potentially fatal complication of rheumatic disordersArchives of Disease in Childhood, 2001
- Perforin Gene Defects in Familial Hemophagocytic LymphohistiocytosisScience, 1999
- Defective Natural Killer Cell Function in Patients with Hemophagocytic Lymphohistiocytosis and in First Degree RelativesPediatric Research, 1998
- Treatment of familial erythrophagocytic lymphohistiocytosis with cyclosporine AThe Journal of Pediatrics, 1997
- Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five casesThe Journal of Pediatrics, 1996
- Characteristic Immune Abnormalities in Hemophagocytic LymphohistiocytosisJournal of Pediatric Hematology/Oncology, 1996
- Intravenous immunoglobulin treatment of experimental T cell-mediated autoimmune disease. Upregulation of T cell proliferation and downregulation of tumor necrosis factor alpha secretion.JCI Insight, 1994