J Korean Med Sci.  2006 Dec;21(6):1124-1127. 10.3346/jkms.2006.21.6.1124.

Macrophage Activation Syndrome in Juvenile Rheumatoid Arthritis Successfully Treated with Cyclosporine A: A Case Report

• You CR
• Kim HR
• Yoon CH
• Lee SH
• Park SH
• Kim HY

• KMID: 1713133
• DOI: http://doi.org/10.3346/jkms.2006.21.6.1124

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).

Keyword

Macrophage activation syndrome; Arthritis, Juvenile Rheumatoid; Cyclosporine

MeSH Terms

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

Figure

• 

  Fig. 1 Radiographic images of hands, feet and hips. (A) Anteroposterior view of both hands shows that periarticular osteopenia and erosions at proximal interphalangeal, metacarpophalangeal, radiocarpal and radioulnar joints. Ankylosis at both carpometacarpal and intercarpal joints. (B) Anteroposterior view of both feet shows the periarticular erosions at metatarsotarsal, tibiotalar, talonavicular and naviculocuneiform joints. (C) Anteroposterior view of both hips shows that extensive pressure erosions in acetabuli and femoral heads, resulting in protrusion of the acetabuli, especially in right hip joint.

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  Fig. 2 Bone marrow aspiration. Cytopathology of bone marrow aspirates shows that histiocytes are markedly increased in number and some of them have active hemophagocytosis (Wright-Giemsa stain, ×400).

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