title: Tumor necrosis factor blockade in the management of children with orphan diseases.
abstract: Tumor necrosis factor (TNF) blockade has been used successfully to treat a number of rheumatic disorders that have a substantial burden of illness. In children, the TNF antagonists are used mainly for the treatment of juvenile idiopathic arthritis (JIA). There are, however, a variety of rare systemic inflammatory diseases, in which TNF blockade appears promising. Preliminary data in adults suggest that several forms of vasculitis appear to be responsive to TNF antagonists-Behcet's disease, polyarteritis nodosa, Wegener granulomatosis, among others. Some of them respond better to infliximab, a chimeric monoclonal anti-TNF antibody, than to etanercept, a recombinant p75 TNF receptor. We describe our limited experience with infliximab in the treatment of three children with rare vasculitic conditions.
authors.affiliation: Department of Pediatrics, Meyer Children's Hospital of Haifa, P.O. Box 9602, Technion, Haifa 31096, Israel. r_brik@rambam.health.gov.il
authors.forename: Riva
authors.initials: R
authors.lastname: Brik
authors.forename: Vardit
authors.initials: V
authors.lastname: Gepstein
authors.forename: Eli
authors.initials: E
authors.lastname: Shahar
authors.forename: Dorit
authors.initials: D
authors.lastname: Goldsher
authors.forename: Drora
authors.initials: D
authors.lastname: Berkovitz
mesh_terms: D000293:Adolescent; D001171:Arthritis, Juvenile / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D010505:Familial Mediterranean Fever / Q000378:metabolism / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D010488:Polyarteritis Nodosa / Q000378:metabolism / Q000628:therapy*; D012507:Sarcoidosis / Q000378:metabolism / Q000628:therapy*; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*; D014657:Vasculitis / Q000628:therapy
chemical_list: D014409:Tumor Necrosis Factor-alpha