J Rheum Dis.  2012 Aug;19(4):225-229. 10.4078/jrd.2012.19.4.225.

A Case of Macropharge Activation Syndrome Successfully Treated with Combination Therapy Including Etanercept

• Sim YS
• Kim HS
• Kim KN

• KMID: 2223076
• DOI: http://doi.org/10.4078/jrd.2012.19.4.225

Abstract

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of childhood systemic inflammatory disorder, primarily systemic onset juvenile rheumatoid arthritis (SoJRA). It is characterized by pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms. The clinical manifestations are caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to cytokine overproduction including tumor necrosis factor-alpha (TNF-alpha). Methylprednisolone pulse therapy and cyclosporine A have made a considerable progress in the treatment of MAS. However, the mortality rate remains high suggesting the need of another therapeutic agent. Several cases of MAS successfully treated with TNF-alpha inhibitor (etanercept) have been reported. We report the first Korean case of MAS successfully treated with combination therapy of corticosteroid, cyclosporine A and etanercept.

Keyword

Macrophage activation syndrome (MAS); Etanercept

MeSH Terms

Arthritis, Juvenile Rheumatoid
Cyclosporine
Hepatic Insufficiency
Immunoglobulin G
Macrophage Activation Syndrome
Macrophages
Methylprednisolone
Neurologic Manifestations
Pancytopenia
Receptors, Tumor Necrosis Factor
T-Lymphocytes
Tumor Necrosis Factor-alpha
Etanercept
Cyclosporine
Immunoglobulin G
Methylprednisolone
Receptors, Tumor Necrosis Factor
Tumor Necrosis Factor-alpha

Figure

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  Figure 1 A typical skin rash of macropharge activation syndrome (MAS).

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  Figure 2 Bone marrow aspirate smears of a macropharge activation syndrome (MAS) demonstrating hemophagocytosis (arrow) (Wright stain, ×1,000).

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