<abstract xmlns="http://www.w3.org/1999/xhtml"><p>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, <italic>with</italic> or <italic>without</italic> 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.</p></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.

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

Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School

Department of Biomedical Sciences, Graduate School of Veterinary Medicine

Romanian Journal of Internal Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Macrophage activation syndrome (MAS), a variant of secondary hemophagocyticlymphohistiocytosis, is a potentially life-threatening complication of inflammatory...

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

Published Online: May 17, 2018

Page range: 117 - 121

Received: Oct 08, 2017

DOI: https://doi.org/10.1515/rjim-2017-0043

Keywords
hemophagocyticlymphohistiocytosis (HLH), intravenous immunoglobulin (IVIG), macrophage activation syndrome (MAS), systemic lupus erythematosus (SLE), tacrolimus (Tac)

© 2018 Natsuki Aoyama-Maeda et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

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

Published Online: May 17, 2018

Page range: 117 - 121

Received: Oct 08, 2017

DOI: https://doi.org/10.1515/rjim-2017-0043

Keywordshemophagocyticlymphohistiocytosis (HLH), intravenous immunoglobulin (IVIG), macrophage activation syndrome (MAS), systemic lupus erythematosus (SLE), tacrolimus (Tac)

© 2018 Natsuki Aoyama-Maeda et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Keywords
hemophagocyticlymphohistiocytosis (HLH), intravenous immunoglobulin (IVIG), macrophage activation syndrome (MAS), systemic lupus erythematosus (SLE), tacrolimus (Tac)