Macrophage activation syndrome in juvenile rheumatoid arthritis successfully treated with cyclosporine A: a case report.
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).
You
Chan-Ran
CR
Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, Korea.
Kim
Hae-Rim
HR
Yoon
Chong-Hyeon
CH
Lee
Sang-Heon
SH
Park
Sung-Hwan
SH
Kim
Ho-Youn
HY
D000328:Adult; D001171:Arthritis, Juvenile / Q000188:drug therapy*; D016572:Cyclosporine / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008206:Lymphatic Diseases / Q000188:drug therapy*; D008262:Macrophage Activation; D008297:Male; D010198:Pancytopenia / Q000188:drug therapy*; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D016572:Cyclosporine