title: Etanercept in the treatment of macrophage activation syndrome.
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.
authors.affiliation: Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Ohio 45229-3039, USA.
authors.forename: S
authors.initials: S
authors.lastname: Prahalad
authors.forename: K E
authors.initials: KE
authors.lastname: Bove
authors.forename: D
authors.initials: D
authors.lastname: Dickens
authors.forename: D J
authors.initials: DJ
authors.lastname: Lovell
authors.forename: A A
authors.initials: AA
authors.lastname: Grom
mesh_terms: 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
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D000068800:Etanercept