A Case of Macropharge Activation Syndrome Successfully Treated with Combination Therapy Including Etanercept.
10.4078/jrd.2012.19.4.225
- Author:
Young Seok SIM
1
;
Hyun Soo KIM
;
Kwang Nam KIM
Author Information
1. Department of Pediatrics, Hallym University Medical Center, Hallym University College of Medicine, Seoul, Korea. rheumatol@korea.com
- Publication Type:Case Report
- Keywords:
Macrophage activation syndrome (MAS);
Etanercept
- MeSH:
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
- From:Journal of Rheumatic Diseases
2012;19(4):225-229
- CountryRepublic of Korea
- Language:Korean
-
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.
