title: Macrophage activation syndrome in children with systemic-onset juvenile chronic arthritis.
abstract: Macrophage activation syndrome (MAS) is a life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile chronic arthritis (SOJCA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study assessed the clinical features of nine MAS events in five children with SOJCA. Nonremitting fever and decreased platelet and white blood cell counts led to a diagnosis of MAS. The urinary beta2-microglobulin (beta2MG) level was a sensitive indicator of MAS. Serum levels of beta2MG and soluble interleukin-2 receptor were also elevated. These biologic markers reflecting hyperactivated cellular immunity are useful indicators of MAS. Four children treated with cyclosporin A (CSP) achieved rapid and complete recovery, but one patient without CSP died due to rapidly progressive respiratory failure. All children treated with CSP responded quickly, and fever abated within 36 h of initiation of treatment. CSP should be added to first-line therapy of MAS.
authors.affiliation: Departmentof Pediatrics, Wakayama Medical University, Kimiidera, Wakayama City, Japan. nami@mail.wakayama-med.ac.jp
authors.forename: Shinji
authors.initials: S
authors.lastname: Kounami
authors.forename: Megumi
authors.initials: M
authors.lastname: Yoshiyama
authors.forename: Keiko
authors.initials: K
authors.lastname: Nakayama
authors.forename: Masumi
authors.initials: M
authors.lastname: Okuda
authors.forename: Shuji
authors.initials: S
authors.lastname: Okuda
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Aoyagi
authors.forename: Norishige
authors.initials: N
authors.lastname: Yoshikawa
mesh_terms: D000293:Adolescent; D001171:Arthritis, Juvenile / Q000097:blood / Q000150:complications* / Q000652:urine; D015415:Biomarkers / Q000097:blood / Q000652:urine; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007223:Infant; D007262:Infusions, Intravenous; D007958:Leukocyte Count; D008262:Macrophage Activation / Q000187:drug effects; D008297:Male; D010976:Platelet Count; D015375:Receptors, Interleukin-2 / Q000097:blood*; D013577:Syndrome; D001613:beta 2-Microglobulin / Q000097:blood* / Q000652:urine*
chemical_list: D015415:Biomarkers; D007166:Immunosuppressive Agents; D015375:Receptors, Interleukin-2; D001613:beta 2-Microglobulin; D016572:Cyclosporine