The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry.
- Girschick H Paediatric and Adolescent Medicine, Perinatal Centre, Clinic for Paediatric and Adolescent Medicine, Vivantes Klinikum Berlin, Germany.
- Finetti M Istituto Giannina Gaslini, Pediatria II, Reumatologia, Genoa.
- Orlando F Istituto Giannina Gaslini, Pediatria II, Reumatologia, Genoa.
- Schalm S Klinikum der Universität, von Haunersches Kinderspital, Munich, Germany.
- Insalaco A Rheumatology Unit, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
- Ganser G Clinic of Paediatric Rheumatology, St. Josef-Stift Hospital, Sendenhorst, Germany.
- Nielsen S Paediatric Rheumatology, Rigshospitalet, Copenhagen, Denmark.
- Herlin T Skejby Sygehus, Aarhus University Hospital, Aarhus, Denmark.
- Koné-Paut I Service de rhumatologie pédiatrique, Le Kremlin-Bicêtre University Hospital, Paris-Sud University, Paris, France.
- Martino S Dipartimento di Scienze Pediatriche e dell'Adolescenza, University of Torino, Torino, Italy.
- Cattalini M Unita di Immunologia e Reumatologia Pediatrica, Clinica Pediatrica dell'Universita di Brescia, Spedali Civili, Brescia, Italy.
- Anton J Paediatric Rheumatology, Hospital Sant Joan de Déu. Universitat de Barcelona, Spain.
- Mohammed Al-Mayouf S Paediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
- Hofer M Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.
- Quartier P IMAGINE Institute, Paris-Descartes University, Necker Children's Hospital, Paris, France.
- Boros C Paediatrics and Reproductive Health, Women's and Children's Hospital, Adelaide, South Australia, Australia.
- Kuemmerle-Deschner J Department of General Paediatrics, University Children's Hospital Tübingen, Tübingen, Germany.
- Pires Marafon D Rheumatology Unit, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
- Alessio M Dipartimento di Pediatria, Università di Napoli Federico II, Naples.
- Schwarz T Section of Paediatric Rheumatology, Children's Hospital, Osteology, Immunology and Infectious Diseases, University of Wuerzburg, Germany.
- Ruperto N Istituto Giannina Gaslini, Pediatria II, Reumatologia, Genoa.
- Martini A Direzione Scientifica, Istituto Giannina Gaslini, Genoa, Italy.
- Jansson A Klinikum der Universität, von Haunersches Kinderspital, Munich, Germany.
- Gattorno M Istituto Giannina Gaslini, Pediatria II, Reumatologia, Genoa.
- 2018-03-30
Published in:
- Rheumatology (Oxford, England). - 2018
SAPHO
autoinflammatory diseases
bisphosphonates
bone inflammation
chronic non-bacterial osteomyelitis
chronic recurrent multifocal osteomyelitis
magnetic resonance imaging
palmoplantar pustulosis
registry
treatment
English
OBJECTIVES
Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.
METHODS
A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved.
RESULTS
Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3%) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88%), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, 2% urticarial rash) and gastrointestinal symptoms (8%). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response.
CONCLUSION
This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations.
Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.
METHODS
A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved.
RESULTS
Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3%) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88%), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, 2% urticarial rash) and gastrointestinal symptoms (8%). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response.
CONCLUSION
This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations.
- Language
-
- English
- Open access status
- bronze
- Identifiers
-
- DOI 10.1093/rheumatology/key058
- PMID 29596638
- Persistent URL
- https://folia.unifr.ch/global/documents/293568
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