Elsevier

The Journal of Pediatrics

Volume 128, Issue 2, February 1996, Pages 275-278
The Journal of Pediatrics

Macrophage activation syndrome in systemic juvenile rheumatoid arthritis successfully treated with cyclosporine,☆☆,,★★

https://doi.org/10.1016/S0022-3476(96)70408-0Get rights and content

Abstract

A macrophage activation syndrome, possibly related to methotrexate toxicity, developed in a boy with systemic juvenile rheumatoid arthritis. Corticosteroid administration was ineffective, whereas a prompt response to cyclosporine was observed. Two months later, Pneumocystis carinii pneumonia developed. (J PEDIATR 1996;128:275-8)

Section snippets

CASE REPORT

A diagnosis of S-JRA5 was made for our patient, a boy, at the age of 12 years. The disease course was marked by persistent activity with corticosteroid-dependent systemic manifestations and severe polyarthritis leading to diffuse joint lesions. One year after onset, treatment with orally administered methotrexate at a dose of 15 mg (7.9 mg/m2) weekly was started. Nonsteroidal antiinflammatory drug therapy consisted of the use of piroxicam. The dose of methotrexate was increased to 30 mg (15.8

DISCUSSION

Macrophage activation syndrome is among the most common causes of death in S-JRA and requires prompt recognition and treatment.1, 2, 6, 7 The mainstay of the management of MAS in JRA is based on high-dose intravenous corticosteroid administration. However, there have been some deaths in the reported series, even among patients treated with massive doses of corticosteroids.3 Therapy with cyclosporine was recently proposed by Stéphan et al.3 as a result of their findings suggesting macrophage

References (12)

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From the Clinica Pediatrica dell'Universita' di Pavia, Istituto di Ricovero e Cura a Carattere Scientifico S. Matteo, Pavia, Italy

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Supported in part by Istituto di Ricovero e Cura a Carattere Scientifico S. Matteo, Pavia, Italy.

Reprint requests: Alberto Martini, MD, Clinica Pediatrica dell'Università di Pavia, Istituto di Ricovero e Cura a Carattere Scientifico S. Matteo, P.le Golgi (2), 27100 Pavia, Italy.

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