Etanercept in the treatment of macrophage activation syndrome.
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.
Prahalad
S
S
Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Ohio 45229-3039, USA.
Bove
K E
KE
Dickens
D
D
Lovell
D J
DJ
Grom
A A
AA
D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage; D001171:Arthritis, Juvenile / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D000068800:Etanercept; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000008:administration & dosage*; D008262:Macrophage Activation / Q000187:drug effects* / Q000502:physiology; D008297:Male; D018124:Receptors, Tumor Necrosis Factor / Q000008:administration & dosage*; D018570:Risk Assessment; D012720:Severity of Illness Index; D012867:Skin / Q000473:pathology; D013577:Syndrome; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D000068800:Etanercept