Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/112035
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Type: Journal article
Title: The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry
Author: Girschick, H.
Finetti, M.
Orlando, F.
Schalm, S.
Insalaco, A.
Ganser, G.
Nielsen, S.
Herlin, T.
Koné-Paut, I.
Martino, S.
Cattalini, M.
Anton, J.
Al-Mayouf, S.
Hofer, M.
Quartier, P.
Boros, C.
Kuemmerle-Deschner, J.
Pires Marafon, D.
Alessio, M.
Schwarz, T.
et al.
Citation: Rheumatology, 2018; 57(7):1203-1211
Publisher: Oxford University Press
Issue Date: 2018
ISSN: 1462-0324
1462-0332
Statement of
Responsibility: 
Hermann Girschick, Martina Finetti, Francesca Orlando, Susanne Schalm, Antonella Insalaco, Gerd Ganser, Susan Nielsen, Troels Herlin, Isabelle Kone, -Paut, Silvana Martino, Marco Cattalini, Jordi Anton, Sulaiman Mohammed Al-Mayouf, Michael Hofer, Pierre Quartier, Christina Boros, Jasmin Kuemmerle-Deschner, Denise Pires Marafon, Maria Alessio, Tobias Schwarz, Nicolino Ruperto, Alberto Martini, Annette Jansson, and Marco Gattorno, for the Paediatric Rheumatology International Trials Organisation, PRINTO, and the Eurofever registry
Abstract: 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.
Keywords: PRINTO and the Eurofever registry
Description: Corrected by: Erratum: The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry, in Rheumatology, key143, https://doi.org/10.1093/rheumatology/key143. This paper was corrected online and in print to correct the affiliations of authors Marco Gattorno and Alberto Martini. The Authors would like to apologize for this error.
Rights: © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.
RMID: 0030085426
DOI: 10.1093/rheumatology/key058
Appears in Collections:Paediatrics publications

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