Successful treatment of macrophage activation syndrome in a patient with dermatomyositis by combination with immunosuppressive therapy and plasmapheresis.

Abstract

Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is mediated by cytokine overproduction from excessive activation of T lymphocytes and macrophages. We present a dermatomyositis patient with MAS, caused by hypercytokinemia. The combination of tacrolimus and plasma exchange therapy was effective in this case for treating MAS. This combination therapy is especially useful for MAS refractory to steroids.

• Authors: Shinjiro Kaieda, Naomi Yoshida, Fumiya Yamashita, Masaki Okamoto, Hiroaki Ida, Tomoaki Hoshino, Takaaki Fukuda
• Journal: Modern rheumatology (Mod Rheumatol) Vol. 25 Issue 6 Pg. 962-6 ( 2015) ISSN: 1439-7609 [Electronic] England
• PMID: 24252010 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunosuppressive Agents
• Tacrolimus

Topics

• Combined Modality Therapy
• Dermatomyositis (complications)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Macrophage Activation Syndrome (complications, drug therapy, therapy)
• Male
• Middle Aged
• Plasmapheresis
• Tacrolimus (therapeutic use)
• Treatment Outcome