Abstract
Macrophage activation syndrome (MAS), a recognized complication of systemic juvenile rheumatoid arthritis (sJRA), has been associated with significant morbidity and mortality. Dysregulation of macrophage-lymphocyte interactions leading to uncontrolled proliferation of highly activated macrophages and massive release of proinflammatory cytokines including tumor necrosis factor-alpha (TNF-alpha) appears to be central to the pathogenesis of this syndrome. Until now the mainstay of therapy has been corticosteroids and cyclosporin A. We describe a patient with MAS and sJRA successfully treated with the anti-TNF agent etanercept. The outcome in this patient suggests etanercept might be an effective therapeutic agent in MAS.
Publication types
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Case Reports
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Anti-Inflammatory Agents, Non-Steroidal / administration & dosage
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Arthritis, Juvenile / complications
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Arthritis, Juvenile / diagnosis*
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Arthritis, Juvenile / drug therapy*
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Biopsy, Needle
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Bone Marrow / pathology
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Child
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Drug Administration Schedule
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Drug Therapy, Combination
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Etanercept
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Follow-Up Studies
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Humans
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Immunoglobulin G / administration & dosage*
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Macrophage Activation / drug effects*
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Macrophage Activation / physiology
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Male
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Receptors, Tumor Necrosis Factor / administration & dosage*
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Risk Assessment
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Severity of Illness Index
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Skin / pathology
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Syndrome
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Treatment Outcome
Substances
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Anti-Inflammatory Agents, Non-Steroidal
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Immunoglobulin G
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Receptors, Tumor Necrosis Factor
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Etanercept