Open Access Research article

Chronic nonbacterial osteomyelitis in childhood: prospective follow-up during the first year of anti-inflammatory treatment

Christine Beck1, Henner Morbach1, Meinrad Beer2, Martin Stenzel23, Dennis Tappe4, Stefan Gattenlöhner5, Ulrich Hofmann6, Peter Raab7 and Hermann J Girschick18*

Author Affiliations

1 Children's Hospital, Section of Paediatric Rheumatology, Osteology, Immunology and Infectious Diseases, University of Würzburg, Josef Schneider Straße 2, 97080 Würzburg, Germany

2 Institute of Radiology, Department of Pediatric Radiology, University of Würzburg, Josef Schneider Straße 2, 97080 Würzburg, Germany

3 University of Jena, Institute of Radiology, Department of Pediatric Radiology, Bachstraße 18, 07743 Jena, Germany

4 Institutes of Hygiene and Microbiology, University of Würzburg, Josef Schneider Straße 2, 97080 Würzburg, Germany

5 Institute of Pathology, University of Würzburg, Josef Schneider Straße 2, 97080 Würzburg, Germany

6 Department of Internal Medicine I, University of Würzburg, Josef Schneider Straße 2, 97080 Würzburg, Germany

7 Department of Orthopedics, Section of Pediatric Orthopedics, Koenig-Ludwig-Haus, Brettreichstraße 11, 97074 Würzburg, Germany

8 Vivantes Children's Hospital, Pediatric Rheumatology, Immunology and Infectious diseases, Landsberger Allee 49, 10249 Berlin-Friedrichshain, Germany

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Arthritis Research & Therapy 2010, 12:R74  doi:10.1186/ar2992

Published: 30 April 2010

Abstract

Introduction

Chronic nonbacterial osteomyelitis (CNO) is an inflammatory disorder of unknown etiology. In children and adolescents CNO predominantly affects the metaphyses of the long bones, but lesions can occur at any site of the skeleton. Prospectively followed cohorts using a standardized protocol in diagnosis and treatment have rarely been reported.

Methods

Thirty-seven children diagnosed with CNO were treated with naproxen continuously for the first 6 months. If assessment at that time revealed progressive disease or no further improvement, sulfasalazine and short-term corticosteroids were added. The aims of our short-term follow-up study were to describe treatment response in detail and to identify potential risk factors for an unfavorable outcome.

Results

Naproxen treatment was highly effective in general, inducing a symptom-free status in 43% of our patients after 6 months. However, four nonsteroidal anti-inflammatory drug (NSAID) partial-responders were additionally treated with sulfasalazine and short-term corticosteroids. The total number of clinical detectable lesions was significantly reduced. Mean disease activity estimated by the patient/physician and the physical aspect of health-related quality of life including functional ability (global assessment/childhood health assessment questionnaire and childhood health assessment questionnaire) and pain improved significantly. Forty-one percent of our patients showed radiological relapses, but 67% of them were clinically silent.

Conclusions

Most children show a favorable clinical course in the first year of anti-inflammatory treatment with NSAIDs. Relapses and new radiological lesions can occur at any time and at any site in the skeleton but may not be clinically symptomatic. Whole-body magnetic resonance imaging proved to be very sensitive for initial and follow-up diagnostics.