Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five cases

doi:10.1016/S0022-3476(96)70160-9

The Journal of Pediatrics

Volume 129, Issue 5, November 1996, Pages 750-754

https://doi.org/10.1016/S0022-3476(96)70160-9 Get rights and content

Abstract

OBJECTIVES: To evaluate the efficacy of cyclosporine A in the treatment of macrophage activation syndrome (MAS) occurring in children with juvenile arthritis. STUDY DESIGN: MAS developed in two boys and three girls with systemic juvenile arthritis (four) and polyarticular juvenile arthritis (one). In three children whose condition was life-threatening, increased parenteral administration of corticosteroids failed to improve their condition; therefore cyclosporine A (2 to 5 mg/kg per day) was added. In two other patients with less severe clinical manifestations, cyclosporine A alone (2 to 8 mg/kg per day) was given. RESULTS: After the introduction of cyclosporine A, rapid improvement was obtained in all patients and apyrexia occurred within 24 to 48 hours. The biologic abnormalities disappeared more slowly (up to 5 weeks for liver enzymes). CONCLUSIONS: These observations underline the usefulness of cyclosporine A in this complication. The use of this drug may circumvent the need for increased doses of corticosteroids in some patients. The mechanism of action of cyclosporine A remains speculative, but these results indicate indirectly that T-helper lymphocytes may play a role in the pathogenesis of MAS. (J Pediatr 1996;129:750-4)

Section snippets

Patient 1

A 4-year-old girl had S-JA at the age of 10 months and was treated with acetylsalicylate, 100 mg/kg per day in divided doses, and prednisone, 0.8 mg/kg per day.

In March 1993 the dosage of prednisone was progressively reduced to 0.5 mg/kg per day, with acceptable control of the disease despite the persistence of polyarthritis. A 6-month trial of therapy with methotrexate, 0.6 mg/kg per day, failed. Varicella developed despite two intramuscular injections of immune globulin (0.3 mg/kg per

DISCUSSION

Macrophage activation syndrome as a complication of juvenile arthritis occurs mainly in children with a systemic onset. A recent review of 44 previously published cases of this syndrome outlines the characteristics of this syndrome.¹² Early recognition is imperative so that appropriate therapy can be initiated immediately, because the mortality rate is high.7, 8, 9, 13 A number of triggers for MAS have been proposed, including gold therapy,4, 9 aspirin, other nonsteroidal antiinflammatory drugs,

Acknowledgements

We are grateful to Marcel Guyot, MD, and Corine Silly, MD, for the information on case 5. Many thanks to Jane Peake, MD, for reading the manuscript.

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^☆: From the Pediatric Rheumatology Unit and the Pediatric Intensive Care Unit, Department of Pediatrics, Hôpital des Enfants-Malades, Université Paris V, Paris, France

^☆☆: Reprint requests: Richard Mouy, MD, Pediatric Rheumatology Unit, Department of Pediatrics, Hôpital des Enfants-Malades, 149 rue de Sèvres, 75730 Paris Cedex 15, France.

^★: 0022-3476/96/$5.00 + 0 9/22/76795

^★★: See commentary, "Macrophage activation syndrome in systemic juvenile rheumatoid arthritis"

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Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five cases☆,☆☆,★,★★

Abstract

Section snippets

Patient 1

DISCUSSION

Acknowledgements

J Pediatr

J Pediatr

J Pediatr

Blood

Immunol Today

Serum enzyme abnormalities in juvenile rheumatoid arthritis

Pediatrics

Aspirin hepatoxicity and disseminated intravascular coagulation

Ann Intern Med

Evidence for intravascular coagulation in systemic onset, but not polyarticular, juvenile rheumatoid arthritis

Arthritis Rheum

Disseminated intravascular coagulation in Still's disease

Semin Arthitis Rheum

Hepatotoxicity with encephalopathy associated with aspirin therapy in juvenile rheumatoid arthritis

J Pediatr

Toxic encephalopathy with hyperammonemia during high dose salicylate therapy

Acta Neurol Scand