Macrophage Activation Syndrome in Juvenile Rheumatoid Arthritis Successfully Treated with Cyclosporine A: A Case Report.
10.3346/jkms.2006.21.6.1124
- Author:
Chan Ran YOU
1
;
Hae Rim KIM
;
Chong Hyeon YOON
;
Sang Heon LEE
;
Sung Hwan PARK
;
Ho Youn KIM
Author Information
1. Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Macrophage activation syndrome;
Arthritis, Juvenile Rheumatoid;
Cyclosporine
- MeSH:
Treatment Outcome;
Pancytopenia/*drug therapy;
Male;
*Macrophage Activation;
Lymphatic Diseases/*drug therapy;
Immunosuppressive Agents/therapeutic use;
Humans;
Cyclosporine/*therapeutic use;
Arthritis, Juvenile Rheumatoid/*drug therapy;
Adult
- From:Journal of Korean Medical Science
2006;21(6):1124-1127
- CountryRepublic of Korea
- Language:English
-
Abstract:
Macrophage activation syndrome (MAS) is one of the serious complications of juvenile rheumatoid arthritis (JRA) and recently, cyclosporine A has been found to be effective in patients with corticosteroid-resistant MAS. A 29-yr-old male was admitted with high fever and jaundice for one month. He was diagnosed as juvenile arthritis 16 yr ago. Physical and laboratory results showed hepatosplenomegaly, high fever, pancytopenia and impaired liver and renal function tests, elevated triglyceride and serum ferritin levels. Bone marrow biopsy showed hyperplasia of histiocytes with active hemophagocytosis. He was diagnosed as MAS associated with juvenile rheumatoid arthritis and managed with high-dose corticosteroids initially, but clinical symptoms and laboratory findings did not improve immediately. Finally, he completely recovered after treatment with cyclosporine A (3 mg/kg/day).