title: Chronic nonbacterial osteomyelitis in children: a retrospective multicenter study.
abstract: BACKGROUND
  To determine the clinical presentation, current treatment and outcome of children with nonbacterial inflammatory bone disease.
  
  
  METHODS
  Retrospective multicenter study of patients entered into the Swiss Pediatric Rheumatology Working Group registry with a diagnosis of chronic nonbacterial osteomyelitis (CNO) and synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome. The charts were reviewed for informations about disease presentation, treatment, course and outcome.
  
  
  RESULTS
  Forty-one children (31 girls and 10 boys) from 6 pediatric hospitals in Switzerland diagnosed between 1995 and 2010 were included in the study. The diagnosis was multifocal CNO (n = 33), unifocal CNO (n = 4) and SAPHO syndrome (n = 4). Mean age at onset of CNO was 9.5 years (range 1.4-15.6) and mean follow-up time was 52 months (range 6-156 months). Most patients (n = 27) had a chronic persistent disease course (>6 months), 8 patients had a course with one or more relapses and 6 patients had only one episode of CNO. Forty nine percent had received at least one course of antibiotics. In 57% treatment with nonsteroidal anti-inflammatory drugs (NSAID) was sufficient to control the disease. Twelve out of 16 children with NSAID failure subsequently received corticosteroids, methotrexate, TNF α inhibitors, bisphosphonates or a combination of these drugs.
  
  
  CONCLUSIONS
  In a multicenter cohort of 41 children 22% started with unifocal lesion with a significant diagnostic delay. A higher proportion presented with chronic persistent disease than with a recurrent form. An osteomyelitis in the pelvic region is significantly associated with other features of juvenile spondylarthritis.
authors.affiliation: Department of Pediatric Rheumatology, Children's Hospital, Kantonsspital Luzern, CH-6000, Luzern 16, Switzerland. daniela.kaiser@luks.ch.
authors.forename: Daniela
authors.initials: D
authors.lastname: Kaiser
authors.affiliation: Department of Pediatric Rheumatology, University Children's Hospital, Berne, Switzerland.
authors.forename: Isabel
authors.initials: I
authors.lastname: Bolt
authors.affiliation: Department of Pediatric Rheumatology, CHUV, Lausanne and HUG, Geneva, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Hofer
authors.affiliation: Department of Pediatric Infectious Diseases, University Children's Hospital Zurich, Zurich, Switzerland.
authors.forename: Christa
authors.initials: C
authors.lastname: Relly
authors.affiliation: Department of Pediatric Rheumatology, Children's Hospital, Aarau, Switzerland.
authors.forename: Gerald
authors.initials: G
authors.lastname: Berthet
authors.affiliation: Department of Pediatric Rheumatology, University Children's Hospital, Basel, Switzerland.
authors.forename: Dieter
authors.initials: D
authors.lastname: Bolz
authors.affiliation: Department of Pediatric Rheumatology, University Children's Hospital, Zurich, Switzerland.
authors.forename: Traudel
authors.initials: T
authors.lastname: Saurenmann
mesh_terms: D020083:Acquired Hyperostosis Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology*; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D015331:Cohort Studies; D004164:Diphosphonates / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D015995:Prevalence; D012189:Retrospective Studies; D013557:Switzerland / Q000453:epidemiology; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D004164:Diphosphonates; D008727:Methotrexate