EFFICACY OF CYCLOSPORINE-A IN THE TREATMENT OF MACROPHAGE ACTIVATION SYNDROME IN JUVENILE ARTHRITIS - REPORT OF 5 CASES

Objectives: To evaluate the efficacy of cyclosporine A in the treatmen t of macrophage activation syndrome (MAS) occurring in children with j uvenile arthritis. Study design: MAS developed in two boys and three g irls with systemic juvenile arthritis (four) and polyarticular juvenil e arthritis (one). In three children whose condition was life-threaten ing, increased parenteral administration of corticosteroids failed to improve their condition; therefore cyclosporine A (2 to 5 mg/kg per da y) was added. In two other patients with less severe clinical manifest ations, cyclosporine A alone (2 to 8 mg/kg per day) was given. Results : After the introduction of cyclosporine A, rapid improvement was obta ined in all patients and apyrexia occurred within 24 to 48 hours, The biologic abnormalities disappeared more slowly (up to 5 weeks for live r enzymes). Conclusions: These observations underline the usefulness o f cyclosporine A in this complication. The use of this drug may circum vent the need for increased doses of corticosteroids in some patients. The mechanism of action of cyclosporine A remains speculative, but th ese results indicate indirectly that T-helper lymphocytes may play a r ole in the pathogenesis of MAS.