Introduction of a novel magnetic resonance imaging-based scoring system for assessing disease activity in children with juvenile dermatomyositis

Objectives: We aimed to develop and assess the reliability of a novel MRI-based scoring system for reporting the severity of MRI findings in children with suspected JDM. Methods: Nine consultant paediatric radiologists independently assessed and scored 40 axial and 30 coronal thigh MR images of children with suspected JDM on two occasions using the juvenile dermatomyositis magnetic resonance Imaging Score (JIS). JIS was calculated for both reads for each plane and each limb, with possible scores ranging from 0 (normal) to 100 (severe). Inter- and intraobserver agreement was calculated using the intraclass correlation coefficient (ICC) and two- and one-way random effects models, respectively. Bland-Altman plots of the difference in JIS against the average JIS were also produced for each rater. Results: Overall, the interobserver reliability and agreement was good-for axial images, JIS ranged from 46.8 to 61.0 [ICC = 0.88 (95% CI: 0.82, 0.92)] for the left limb and 47.9-61.4 [ICC = 0.87 (95% CI: 0.81, 0.92)] for the right limb. For coronal images, JIS ranged from 56.7 to 65.1 [ICC = 0.90 (95% CI: 0.85, 0.95)] for the left limb and 55.7 to 66.8 [ICC = 0.90 (95% CI: 0.84, 0.94)] for the right limb. The intraobserver reliability and agreement was good, with ICC ranging from 0.90 to 0.94. Conclusion: JIS is a semi-objective scoring system with potential to serve as a reliable biomarker of disease severity and response to therapeutic interventions in children with JDM

Classification and management strategies for paediatric chronic nonbacterial osteomyelitis and chronic recurrent multifocal osteomyelitis.

IntroductionChronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone disease that most commonly affects children and adolescents causing significant pain and damage to bones. The absence of diagnostic criteria and biomarkers, an incomplete understanding of the molecular pathophysiology, and lack of evidence from randomized and controlled trials make the diagnosis and care challenging.Areas coveredThis review provides an overview of the clinical and epidemiological features of CNO and displays diagnostic challenges and how they can be addressed following strategies used internationally and by the authors. It summarizes the molecular pathophysiology, including pathological activation of the NLRP3 inflammasome and IL-1 secretion, and how these observations can inform future treatment strategies. Finally, it provides a summary of ongoing initiatives aiming at classification criteria (ACR/EULAR) and outcome measures (OMERACT) that will enable the generation of evidence through clinical trials.Expert opinionScientific efforts have linked molecular mechanisms to cytokine dysregulation in CNO, thereby delivering arguments for cytokine blocking strategies. Recent and ongoing collaborative international efforts are providing the basis to move toward clinical trials and target directed treatments for CNO that find approval by regulatory agencies