Lisa Gamalero1, Alexandre Belot2, Mojca Zajc Avramovic3, Teresa Giani4, Giovanni Filocamo5, Sandesh Guleria6, Giovanna Ferrara7, Francesca Minoia5, Michael Hofer8, Jean Paul Larbre9, Melanie Aureal9, Natasa Toplak3, Tadej Avcin3, Cecilia Beatrice Chighizola10, Rolando Cimaz11. 1. University of Udine, Italy. 2. Paediatric Rheumatology, Nephrology, Dermatology Unit, National Reference Centre for Rheumatism and Systemic Autoimmune Diseases in Children RAISE, Hospices Civils de Lyon, France. 3. Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Slovenia. 4. Rheumatology Unit, Azienda Ospedaliero Universitaria Meyer, Florence, and University of Siena, Italy. 5. Paediatric Rheumatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. 6. Paediatric Allergy Immunology Unit, Advanced Paediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 7. Santa Maria Annunziata Hospital, ASL Toscana Centro, Florence, Italy. 8. Paediatric Unit, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland. 9. Rheumatology Unit, Hopital Lyon Sud, Lyon, France. 10. Experimental Laboratory of Immunological and Rheumatologic Researches, Immunology and Rheumatology Unit, San Luca Hospital, Istituto Auxologico Italiano, IRCCS, Milan, Italy. c.chighizola@auxologico.it. 11. Department of Clinical Sciences and Community Health, University of Milan, and Paediatric Rheumatology Unit, ASST G. Pini & CTO, Milan, Italy.
Abstract
OBJECTIVES: Chronic non-bacterial osteomyelitis (CNO) is a rare non-infectious bone inflammatory disorder; when multifocal, it is referred to as Chronic Recurrent Multifocal Osteomyelitis (CRMO). This study evaluates the demographic, clinical and radiological characteristics of a multi-centre cohort of patients with CNO/CRMO. METHODS: Demographic and clinical data of patients with an established diagnosis of CNO/CRMO followed at paediatric rheumatology centres across Europe (Italy, France, Slovenia) and India were retrospectively collected. RESULTS: There were no demographic differences across countries, but time to diagnosis was significantly longer in India (p=0.041). Pain was almost invariably present at disease onset; functional impairment was more frequent among Italian and Slovenian patients (p=0.001). The number of sites of bone involvement was similar between genders and countries, with long bone metaphises being the most common site. Raised acute phase reactants, detected in >50% of patients, were not associated with clinical manifestations or response to treatment. Comorbidities, evinced in 37% of patients, were equally distributed between genders and nationalities. Imaging approach was similar across countries, without any association between radiological findings and clinical manifestations. NSAIDs were almost invariably used as first-line treatment, but response rate was significantly lower in Italy (p=0.02). Methotrexate was used in 28% of case, with an overall rate of response of 82%. Health conditions and rate of permanent deformities were similar across different countries. CONCLUSIONS: The differences in clinical presentation, radiological features and response to treatment described in this multinational cohort of CNO/CRMO might provide novel insights into this still elusive disease.
OBJECTIVES: Chronic non-bacterial osteomyelitis (CNO) is a rare non-infectious bone inflammatory disorder; when multifocal, it is referred to as Chronic Recurrent Multifocal Osteomyelitis (CRMO). This study evaluates the demographic, clinical and radiological characteristics of a multi-centre cohort of patients with CNO/CRMO. METHODS: Demographic and clinical data of patients with an established diagnosis of CNO/CRMO followed at paediatric rheumatology centres across Europe (Italy, France, Slovenia) and India were retrospectively collected. RESULTS: There were no demographic differences across countries, but time to diagnosis was significantly longer in India (p=0.041). Pain was almost invariably present at disease onset; functional impairment was more frequent among Italian and Slovenian patients (p=0.001). The number of sites of bone involvement was similar between genders and countries, with long bone metaphises being the most common site. Raised acute phase reactants, detected in >50% of patients, were not associated with clinical manifestations or response to treatment. Comorbidities, evinced in 37% of patients, were equally distributed between genders and nationalities. Imaging approach was similar across countries, without any association between radiological findings and clinical manifestations. NSAIDs were almost invariably used as first-line treatment, but response rate was significantly lower in Italy (p=0.02). Methotrexate was used in 28% of case, with an overall rate of response of 82%. Health conditions and rate of permanent deformities were similar across different countries. CONCLUSIONS: The differences in clinical presentation, radiological features and response to treatment described in this multinational cohort of CNO/CRMO might provide novel insights into this still elusive disease.