1 - Área de Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
2 - Unidade de Reumatologia Pediátrica, Hospital Pediátrico de Coimbra, Centro Hospitalar Universitário de Coimbra
3 - Unidade de Reumatologia Pediátrica, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte
4 - Unidade de Reumatologia Pediátrica, Centro Hospitalar e Universitário de S. João
5 - Consulta de Reumatologia Pediátrica, Centro Hospitalar do Médio Ave
6 - Consulta de Reumatologia Pediátrica, Serviço de Pediatria, Centro Hospitalar de Vila Nova de Gaia/Espinho
7 - Consulta Reumatologia Pediátrica, Hospital Prof Dr. Fernando Fonseca, Lisboa
8 - Unidade de Infeciologia Pediátrica, Área de Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
9 - Unidade de Reumatologia Pediátrica; Área de Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- Reunião nacional: Fórum de Reumatologia Pediátrica 2019, comunicação oral
Introduction: Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone disorder characterized by sterile bone lesions.
Objective: The aim of this study is to determine the clinical presentation, current treatment and outcome of children with nonbacterial inflammatory bone disease in Portugal.
Methods: Review of medical records of patients with CNO < 2019 with symptoms onset < 18 years old. Centers were contacted by the Portuguese Pediatric Rheumatology Network.. Collected data included: demographic, clinical, laboratory, imaging, treatment, course and outcome. We segregate our cohort based on unifocal vs. multifocal disease and year of onset of symptoms.
Results: 57 patients, from 8 paediatric rheumatology centres were included. The disease was more common in girls (57,9%), mean age at onset was 10,1 years (SD 3,68). There was an overall delay in diagnosis of 14,4 months, but after 2009 78,8% of patients had a time to diagnosis < 1 year vs. 21% before 2009 . Bone pain was present in 98,2% and swelling/erythema in 57,9%. Multifocal bone involvement was more frequent (63,2%) than unifocal (36,8%), with a median of 2 affected bones, 27,8% symmetrical. Clavicle was the most common site in unifocal disease (38,1. We found no significant differences between unifocal and multifocal group (inflammatory markets, antibiotic therapy, clinical remission), except that 66,7% of unifocal disease group responded to NSAID vs. 33,3% in multifocal group (p< 0,05). Skin lesions were found in 24,6% of our cohort. A whole-body study (MRI or Bone Scan) was performed in 96,4% of patients founding asymptomatic