Abstract
Hemophagocytic syndrome has been reported to be associated with systemic lupus erythematosus. A 25-year-old woman with systemic lupus erythematosus developed hemophagocytic syndrome that was refractory to the combination therapy with high-dose corticosteroid, cyclosporine, and high-dose intravenous immunoglobulin, and successfully treated with the tumor necrosis factor inhibitor, etanercept. This case report provided the first observation that etanercept may be useful for the treatment of hemophagocytic syndrome associated with systemic lupus erythematosus refractory to conventional therapy.
Similar content being viewed by others
References
Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Int J Hematol. 2000;72:1–11.
Osugi Y, Hara J, Tagawa S, et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood. 1997;89:4100–3.
Billiau AD, Roskams T, Van Damme-Lombaerts R, Matthys P, Wouters C. Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alpha-producing macrophages. Blood. 2005;105:1648–51.
Wong KF, Hui PK, Chan JK, Chan YW, Ha SY. The acute lupus hemophagocytic syndrome. Ann Intern Med. 1991;114:387–90.
Mouy R, Stephan JL, Pillet P, Haddad E, Hubert P, Prieur AM. Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: report of five cases. J Pediatr. 1996;129:750–4.
Emmenegger U, Frey U, Reimers A, Fux C, Semela D, Cottagnoud P, Spaeth PJ, Neftel KA. Hyperferritinema as indicator for intravenous immunoglobulin treatment in reactive macrophage activation syndromes. Am J Hematol. 2001;68:4–10.
Moon UY, Park SJ, Oh ST, Kim WU, Park SH, Lee SH, Cho CS, Kim HY, Lee WK, Lee SK. Patients with systemic lupus erythematosus have abnormally elevated Epstein-Barr virus load in blood. Arthritis Res Ther. 2004;6:295–302.
Balandraud N, Meynard JB, Auger I, Sovran H, Mugnier B, Reviron D, Roudier J, Roudier C. Epstein-Barr virus load in the peripheral blood of patients with rheumatoid arthritis: accurate quantification using real-time polymerase chain reaction. Arthritis Rheum. 2003;48:1223–8.
Ravelli A, Caria MC, Buratti S, Malattia C, Temporini F, Martini A. Methotrexate as a possible trigger of macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol. 2001;28:865–7.
Henzan T, Nagafuji K, Tsukamoto H, Miyamoto T, Gondo H, Imashuku S, Harada M. Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis. Am J Hematol. 2006;81:59–61.
Cortis E, Insalaco A. Macrophage activation syndrome in juvenile idiopathic arthritis. Acta Paediatr Suppl. 2006;95:38–41.
Arlet JB, Le TH, Marinho A, Amoura Z, Wechsler B, Papo T, Piette JC. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis. 2006;65:1596–601.
Eriksson C, Engstrand S, Sundqvist KG, Rantapaa-Dahlqvist S. Autoantibody formation in patients with rheumatoid arthritis treated with anti-TNF alpha. Ann Rheum Dis. 2005;64:403–7.
Shakoor N, Michalska M, Harris CA, Block JA. Drug-induced systemic lupus erythematosus associated with etanercept therapy. Lancet. 2002;359:579–80.
De Ryche L, Baeten D, Kruithof E, van den Bosch F, Veys EM, De Keyser F. Infliximab, but not etanercept, induces IgM anti-double-stranded DNA autoantibodies as main antinuclear reactivity: biologic and clinical implications in autoimmune arthritis. Arthritis Rheum. 2005;52:2192–201.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Takahashi, N., Naniwa, T. & Banno, S. Successful use of etanercept in the treatment of acute lupus hemophagocytic syndrome. Mod Rheumatol 18, 72–75 (2008). https://doi.org/10.1007/s10165-007-0006-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10165-007-0006-z