Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five cases

1996 ◽  
Vol 129 (5) ◽  
pp. 750-754 ◽  
Author(s):  
Richard Mouy ◽  
Jean-Louis Stephan ◽  
Pascal Pillet ◽  
Elie Haddad ◽  
Philippe Hubert ◽  
...  
Keyword(s):  
Cyclosporine A ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Juvenile Arthritis ◽  
Activation Syndrome

scholarly journals Macrophage activation syndrome associated with systemic lupus erythematosus treated successfully with the combination of steroid pulse, immunoglobulin and tacrolimus

2018 ◽  
Vol 56 (2) ◽  
pp. 117-121
Author(s):  
Natsuki Aoyama-Maeda ◽  
Taro Horino ◽  
Osamu Ichii ◽  
Yoshio Terada
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Cyclosporine A ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Treatment Protocol ◽  
Systemic Lupus ◽  
Intravenous Immunoglobulin Therapy ◽  
Steroid Pulse ◽  
Activation Syndrome

Abstract Macrophage activation syndrome (MAS), a variant of secondary hemophagocyticlymphohistiocytosis, is a potentially life-threatening complication of inflammatory and autoimmune diseases. We present a case of MAS as a rare manifestation of systemic lupus erythematosus. Although initial treatment with corticosteroid, with or without cyclosporine A, is justified in patients with MAS, evidence regarding the effectiveness of this treatment protocol remains to be clarified. Our patient was successfully treated with a combination of intravenous immunoglobulin therapy and intravenous methyl predonisolone pulse therapy, which was followed by a course of oral prednisolone and oral tacrolimus. Based on our experience, we propose tacrolimus to provide a more useful adjuvant treatment to corticosteroid therapy than cyclosporine A.

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