ABSTRACT
Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease that primarily affects children and adolescents. CNO is associated with pain, bone swelling, deformity, and fractures. Its pathophysiology is characterized by increased inflammasome assembly and imbalanced expression of cytokines. Treatment is currently based on personal experience, case series and resulting expert recommendations. Randomized controlled trials (RCTs) have not been initiated because of the rarity of CNO, expired patent protection of some medications, and the absence of agreed outcome measures. An international group of fourteen CNO experts and two patient/parent representatives was assembled to generate consensus to inform and conduct future RCTs. The exercise delivered consensus inclusion and exclusion criteria, patent protected (excludes TNF inhibitors) treatments of immediate interest (biological DMARDs targeting IL-1 and IL-17), primary (improvement of pain; physician global assessment) and secondary endpoints (improved MRI; improved PedCNO score which includes physician and patient global scores) for future RCTs in CNO.
Subject(s)
Antirheumatic Agents , Osteomyelitis , Child , Adolescent , Humans , Consensus , Cytokines , Antirheumatic Agents/therapeutic use , Osteomyelitis/drug therapy , Pain/complications , Pain/drug therapy , Chronic DiseaseABSTRACT
OBJECTIVES: To correlate clinical findings of Non-bacterial Osteitis (NBO) with whole-body MRI (WB-MRI) findings and determine a radiologic index for NBO (RINBO) which allows standardized reporting of WB-MRI. METHODS AND MATERIALS: In a prospective study, 40 patients with diagnosis of NBO underwent clinical examination and WB-MRI in which STIR- and T1- weighted images were assessed for NBO-typical lesions. Parameters of interest for RINBO were: number of radiologically active lesions (RAL), size of the patients' maximum RAL presence of extramedullary and spinal involvement. Results were tested for statistical agreement of clinical and MR-based lesion detection. RINBO was tested for correlation with clinical activity. RESULTS: 62/95 clinically/radiologically active lesions were found in 30/33 patients. In 45 % of the cohort, more active lesions were detected by WB-MRI than by clinical examination. RINBO was a significant predictor for the presence of clinically active lesions. CONCLUSION: WB-MRI is a powerful diagnostic tool for patients with NBO which can reveal asymptomatic disease activity. With RINBO a standardized evaluation approach is proposed which helps assessing radiologic disease burden and predicts clinical disease activity. KEY POINTS: ⢠Whole body MRI is a powerful diagnostic tool for patients with non-bacterial Osteitis. ⢠Whole body MRI can reveal asymptomatic disease activity. ⢠The radiologic index RINBO offers a standardized evaluation approach.
Subject(s)
Osteitis/pathology , Adolescent , Adult , Biomarkers/metabolism , Child , Female , Humans , Magnetic Resonance Imaging/methods , Male , Middle Aged , Physical Examination/methods , Prospective Studies , Whole Body Imaging/methods , Young AdultABSTRACT
Moving from child- to adult-centred services (transition) is a challenge for patients and health care professionals. Every second adolescent with a rheumatic disease will still have active disease that requires treatment when they are adults. A third of these patients do not arrive in adult-centered care, a consequence that is accompanied by negative consequences on the individual course of disease and contributes to socioeconomical costs. The present article describes the current situation: barriers for successful transfer and transition models are discussed. Transitional care and research must be improved in Germany.