Hermann Girschick1,2, Martina Finetti3, Francesca Orlando3,4, Susanne Schalm5, Antonella Insalaco6, Gerd Ganser7, Susan Nielsen8, Troels Herlin9, Isabelle Koné-Paut10, Silvana Martino11, Marco Cattalini12, Jordi Anton13, Sulaiman Mohammed Al-Mayouf14, Michael Hofer15, Pierre Quartier16, Christina Boros17, Jasmin Kuemmerle-Deschner18, Denise Pires Marafon6, Maria Alessio4, Tobias Schwarz2,7, Nicolino Ruperto3, Alberto Martini19, Annette Jansson5, Marco Gattorno3. 1. Paediatric and Adolescent Medicine, Perinatal Centre, Clinic for Paediatric and Adolescent Medicine, Vivantes Klinikum Berlin, Germany. 2. Section of Paediatric Rheumatology, Children's Hospital, Osteology, Immunology and Infectious Diseases, University of Wuerzburg, Germany. 3. Istituto Giannina Gaslini, Pediatria II, Reumatologia, Genoa. 4. Dipartimento di Pediatria, Università di Napoli Federico II, Naples. 5. Klinikum der Universität, von Haunersches Kinderspital, Munich, Germany. 6. Rheumatology Unit, Ospedale Pediatrico Bambino Gesù, Rome, Italy. 7. Clinic of Paediatric Rheumatology, St. Josef-Stift Hospital, Sendenhorst, Germany. 8. Paediatric Rheumatology, Rigshospitalet, Copenhagen, Denmark. 9. Skejby Sygehus, Aarhus University Hospital, Aarhus, Denmark. 10. Service de rhumatologie pédiatrique, Le Kremlin-Bicêtre University Hospital, Paris-Sud University, Paris, France. 11. Dipartimento di Scienze Pediatriche e dell'Adolescenza, University of Torino, Torino, Italy. 12. Unita di Immunologia e Reumatologia Pediatrica, Clinica Pediatrica dell'Universita di Brescia, Spedali Civili, Brescia, Italy. 13. Paediatric Rheumatology, Hospital Sant Joan de Déu. Universitat de Barcelona, Spain. 14. Paediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. 15. Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland. 16. IMAGINE Institute, Paris-Descartes University, Necker Children's Hospital, Paris, France. 17. Paediatrics and Reproductive Health, Women's and Children's Hospital, Adelaide, South Australia, Australia. 18. Department of General Paediatrics, University Children's Hospital Tübingen, Tübingen, Germany. 19. Direzione Scientifica, Istituto Giannina Gaslini, Genoa, Italy.
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.
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.
Authors: Ashna Ie Ramautar; Natasha M Appelman-Dijkstra; Shannon Lakerveld; Marielle A Schroijen; Marieke Snel; Elizabeth M Winter; Neveen At Hamdy Journal: JBMR Plus Date: 2021-04-10