Sviluppo e validazione di nuove misure di valutazione dello stato di malattia nella dermatomiosite giovanile

[da inserire

Sense of Coherence (SOC) of Italian healthcare workers during the Covid-19 pandemic: analysis of associated factors

Introduction: The COVID-19 pandemic has posed significant challenges for healthcare workers worldwide, potentially affecting their sense of coherence (SOC) and overall well-being. This study aimed to identify factors associated with different levels of SOC among healthcare workers. Methods: A cross-sectional study was conducted among 628 healthcare workers. SOC scores were assessed using a standardized questionnaire, and data on demographic characteristics, length of service, geographic location, and pandemic-related experiences were collected. Statistical analyses were performed to identify associations between these factors and SOC. Results: Healthcare workers had a lower average SOC score (mean: 57.1) compared to the national average in Italy (mean: 60.3). Younger age and shorter length of service were associated with a higher risk of low SOC (p<0.0001). Healthcare workers in the northwestern regions of Italy had an increased risk of low SOC compared to their counterparts in the northeastern regions (p=0.0336). Adverse pandemic-related experiences and worsening social relationships were also associated with a higher risk of low SOC (p<0.0001). Conclusions: This study highlights the unique challenges and stressors faced by healthcare workers during the COVID-19 pandemic and their impact on SOC. Age, length of service, geographic location, and social were significant factors influencing SOC levels. Targeted interventions are needed to enhance SOC and well-being, particularly for younger and newly employed healthcare workers. Strategies promoting social connections, work-life balance, and psychological support services are crucial to support healthcare workers' resilience and coping abilities

Evaluation of 21-Numbered Circle and 10-Centimeter Horizontal Line Visual Analog Scales for Physician and Parent Subjective Ratings in Juvenile Idiopathic Arthritis

Objective.To evaluate the measurement properties of 21-numbered circle visual analog scales (VAS) and traditional 10-cm horizontal line VAS for physician and parent subjective ratings in children with juvenile idiopathic arthritis (JIA).Methods.We studied 2 patient samples in whom physician global rating of overall disease activity, parent global rating of the child's overall well-being, and parent rating of intensity of child's pain were performed using traditional 10-cm horizontal line VAS (n = 397) or 21-numbered circle VAS (n = 471). The measurement performances of the 2 VAS formats were examined by assessing construct validity, score distribution, responsiveness to change over time, and minimal clinically important difference (MCID).Results.Most Spearman correlations with other JIA outcome measures yielded by 21-numbered circle VAS were greater than those obtained with 10-cm horizontal line VAS, revealing that the circle VAS format has better construct validity. Ceiling effects (i.e., score = 0) for physician and parent global ratings were 43.7% and 32.9%, respectively, on 21-numbered circle VAS, and 31.6% and 35.3%, respectively, on 10-cm horizontal line VAS. Responsiveness of 21-numbered circle VAS was good (standardized response mean > 0.8) or moderate (standardized response mean > 0.6) among patients classified as improved or worsened, respectively, by the physician or the parent. Overall, MCID values for 21-numbered circle VAS tended to be greater for worsening than for improvement.Conclusion.The 21-numbered circle VAS are a suitable alternative to the 10-cm horizontal line VAS and may facilitate incorporation of physician and parent subjective ratings in standard clinical practice

Multi-centre national audit of juvenile localised scleroderma:Describing current UK practice in disease assessment and management

OBJECTIVE: To describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations. METHODS: Patients were included if they were diagnosed with JLS and were under the care of paediatric rheumatology between 04/2015-04/2016. Retrospective data was collected in eleven UK centres using a standardised proforma and collated centrally. RESULTS: 149 patients were included with a median age of 12.5 years. The outcome measures recommended by the PRES scleroderma working party were not utilised widely. The localised scleroderma cutaneous assessment tool was only used in 37.6% of patients. Screening for extracutaneous manifestations did not meet recommendations that patients with head involvement have regular screening for uveitis and baseline magnetic resonance imaging (MRI) brain: only 38.5% of these patients were ever screened for uveitis; 71.2% had a MRI brain. Systemic treatment with disease-modifying anti-rheumatic drugs (DMARDs) or biologics was widely used (96.0%). In keeping with the recommendations, 95.5% of patients were treated with methotrexate as first-line therapy. 82.6% received systemic corticosteroids and 34.2% of patients required two or more DMARDs/biologics, highlighting the significant treatment burden. Second-line treatment was mycophenolate mofetil in 89.5%. CONCLUSION: There is wide variation in assessment and screening of patients with JLS but a consistent approach to systemic treatment within UK paediatric rheumatology. Improved awareness of PRES recommendations is required to ensure standardised care. As recommendations are based on low level evidence and consensus opinion, further studies are needed to better define outcome measures and treatment regimens for JLS

Innovative Research Design to Meet the Challenges of Clinical Trials for Juvenile Dermatomyositis

Purpose of Review: This paper aims to provide a summary of the recent therapeutic advances and the latest research on outcome measures for clinical trials in juvenile dermatomyositis (JDM). Recent Findings: Recent randomized controlled trials (RCTs) have demonstrated the superiority of the combination of prednisone with methotrexate over other conventional therapies and the potential effectiveness of rituximab in refractory cases. A multinational project has led to develop new criteria for the definition of minimal, moderate, and major improvement in future JDM clinical trials. This effort has been paralleled by the establishment of criteria for clinically inactive disease. The validation of the first composite disease activity score for JDM is in progress. Summary: The new outcome measures will increase the reliability of assessment of clinical response in JDM clinical trials and foster future multinational RCTs aimed to investigate novel treatment strategies for refractory forms of JDM

Experience with the use of mycophenolate mofetil in juvenile idiopathic inflammatory myopathies

Objective The objective of this study was to evaluate the efficacy and safety of MMF in juvenile idiopathic inflammatory myopathies (JIIMs). Methods Patients diagnosed with JIIM and treated with MMF enrolled in the Juvenile Dermatomyositis Research Group (JDRG) in the UK or followed at the Giannina Gaslini Institute in Genoa, Italy, were included. The following information was collected retrospectively at MMF initiation, at 3, 6 and 12 months after treatment start, and at last follow-up visit: clinical manifestations, laboratory data, physicians' subjective assessment of disease activity, standardized outcome measures of muscle strength/endurance, cutaneous disease activity, physical function, global disease activity, cumulative damage, and ongoing treatment. Results Of the 29 patients included, 23 had juvenile DM and 6 had overlap myositis. During administration of MMF, improvement in measures of muscle strength, skin disease activity, and overall disease activity was seen, with an increase in the frequency of normal scores for Manual Muscle Test-8 from 50.0% to 83.3%, Childhood Myositis Activity Score from 53.5% to 88.9%, muscle component of DAS from 55.2% to 84.2%, skin component of DAS from 31.0% to 42.1%, visual analogue scale for skin disease activity from 25.0% to 47.4%, and visual analogue scale for overall disease activity from 7.1% to 42.1%. The number of patients with inactive disease increased from 10.3% at baseline to 68.5% at last follow-up. CS dose was significantly reduced, from 0.3 to 0.1 mg/kg/day. No relevant side effects were reported. Conclusion Our experience suggests that MMF is a valuable therapeutic option for the management of JIIM

Exercise Capacity and Cardiorespiratory Fitness in Children with Congenital Heart Diseases: A Proposal for an Adapted NYHA Classification

Objective: To propose and evaluate an adapted NYHA classification for children with congenital heart disease (CHD) as a feasible clinical tool for classifying patients' fitness, cardiorespiratory efficiency and functional limitations during their ordinary daily activities, which are also characterized by vigorous and competitive physical exercise among peers. Methods: This cross-sectional investigation analyzed 332 patients (13.1 ± 3.01 y/o) who underwent surgical repair of CHD and performed Cardiopulmonary Exercise Testing (CPET). Patients were divided into NYHA class I, IIA and IIB by specific questioning regarding functional limitation and performance compared to peers and at strenuous intensity. Class IIA was characterized by slight exercise limitation only for strenuous/competitive activities, whereas IIB for already ordinary physical activities. These NYHA classes were compared with maximal CPET on treadmill. Results: Patients' exercise capacity (exercise time, METs), aerobic capacity (VO2peak) and chronotropic response were found progressively impaired when NYHA class I was compared with IIA and IIB. Indeed, ventilatory-perfusion mismatch (PETCO2, VE/VCO2) significantly worsened from NYHA class I to IIA, while no difference was found between IIA and IIB. Conclusion: This adapted NYHA-CHD classification could allow regular functional evaluations and accurate assessments by clinicians, leading to facilitated clinical management and timely medical interventions

A prediction rule for polyarticular extension in oligoarticular-onset juvenile idiopathic arthritis

To search for predictors of polyarticular extension in children with oligoarticular-onset juvenile idiopathic arthritis (JIA) and to develop a prediction model for an extended course

Analysis of arthritis flares after achievement of inactive disease with methotrexate monotherapy in juvenile idiopathic arthritis

To investigate the frequency of arthritis flare and factors affecting occurrence of flare in children with juvenile idiopathic arthritis (JIA) who achieved inactive disease (ID) with methotrexate (MTX) monotherapy