Paediatric Rheumatology Fails to Meet Current Benchmarks, a Call for Health Equity for Children Living with Juvenile Idiopathic Arthritis, Using Digital Health Technologies.

PURPOSE OF REVIEW: This critical review begins by presenting the history of Juvenile Idiopathic Arthritis (JIA) management. To move the conversation forward in addressing the current shortcomings that exist in the clinical management of children living with JIA, we argue that to date, the advancement of successful treatments for JIA has been historically slow. Factors implicated in this situation include a lack of rigorous research, JIA being considered a rare disease, and JIA's idiopathic and complex pathophysiology. RECENT FINDINGS: Despite the well-intended legislative changes to increase paediatric research, and the major advancements seen in molecular medicine over the last 30 years, globally, paediatric rheumatology services are still failing to meet the current benchmarks of best practice. Provoking questions on how the longstanding health care disparities of poor access and delayed treatment for children living with JIA can be improved, to improve healthcare outcomes. Globally, paediatric rheumatology services are failing to meet the current benchmarks of best practice. Raising awareness of the barriers hindering JIA management is the first step in reducing the current health inequalities experienced by children living with JIA. Action must be taken now, to train and well-equip the paediatric rheumatology interdisciplinary workforce. We propose, a resource-efficient way to improve the quality of care provided could be achieved by embedding digital health into clinical practice, to create an integrative care model between the children, general practice and the paediatric rheumatology team. To improve fragmented service delivery and the coordination of interdisciplinary care, across the healthcare system.

Effects of a home-based exercise combined yoga and resistance training in enthesitis-related arthritis in China: study protocol for a randomised controlled trial.

INTRODUCTION: Enthesitis-related arthritis (ERA) is a category of juvenile idiopathic arthritis (JIA). The complications of JIA include pain, muscle weakness, limited movement and worsening quality of life. Yoga is an effective exercise therapy for rheumatoid arthritis and may have similar benefits for JIA. Considering the limitation of yoga for strengthening muscles, combined yoga and resistance training (CYRT) may compensate for the shortcomings and provide more benefits for JIA patients. Despite this, there is currently a lack of studies investigating the effectiveness of CYRT for JIA patients. Due to the inaccessibility of traditional exercise therapy, home-based exercise is needed. The study aims to assess the effectiveness of home-based CYRT on JIA. METHODS AND ANALYSIS: This is a 12-week randomised single-blind controlled trial study. 60 patients with ERA will be randomised into two groups: the home-based exercise group (HBE) and the health education (HE) group. The HBE group (n=30) will perform the CYRT programme 3 times a week at home for 12 weeks and receive HE. The HE group (n=30) will only receive HE. The outcomes include primary outcome (pain levels) and secondary outcomes (lower limb muscle strength, motion range of joint, aerobic fitness, function ability, fatigue levels, mental health, quality of life and blood biomarkers). The assessments will be conducted at baseline, postintervention (12 weeks) and follow-up (24 weeks). Data will be analysed by intention to treat. ETHICS AND DISSEMINATION: This study has been approved by the Ethics Committee of Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine in December 2023 (approval no. XHEC-C-2023-059-3). This study will require informed consent from all subjects and guardians of children under 18 years of age. The findings will be published in a peer-reviewed journal and international conferences. TRIAL REGISTRATION NUMBER: ChiCTR2300073446.

The effects of inspiratory muscle training on cardiorespiratory functions in juvenile idiopathic arthritis: A randomized controlled trial.

INTRODUCTION: Although inspiratory muscle training (IMT) has proven effective in adult rheumatic diseases, its impact on juvenile idiopathic arthritis (JIA) remains unexplored. The present study aimed to investigate the effects of IMT in children with JIA. METHODS: Thirty-three children (13-18 years) with JIA were divided into two groups as exercise (n = 17) and control (n = 16). The exercise group performed IMT at home daily for 8 weeks. The initial IMT load was set as 60% of maximal inspiratory pressure (PImax ) and increased by %10 of the initial load every 2 weeks. The control group received no additional intervention. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1 ), FVC/FEV1 , PImax , and maximal expiratory pressure (PEmax ) were evaluated. Peak oxygen consumption (VO2max ), metabolic equivalents (METs), and maximal heart rate were measured with cardiopulmonary exercise test. Functional capacity and quality of life were assessed with 6-min walk distance and Pediatric Quality of Life Inventory 3.0 Arthritis Module. All participants were evaluated at baseline and post-treatment. RESULTS: FVC ( ↑ 0.20 (95% CI: 0.07/0.32) liters), FEV1 ( ↑ 0.14 (95% CI: 0.02/0.25) liters), PImax (↑19.11 (95% CI: 9.52/28.71) cmH2 O), PEmax (↑12.41 (95% CI: 3.09/21.72) cmH2 O), VO2peak (↑158.29 (95% CI: 63.85/252.73) ml/min), and METs (↑0.92 (95% CI: 0.34/1.49) [ml/kg/min]) significantly improved only in the exercise group (p < .05). The difference over time in FVC, FEV1 , PImax , VO2peak , and METs were significantly higher in exercise group compared to control group (p < .05). CONCLUSIONS: IMT seems to be an effective option for improving respiratory functions and aerobic exercise capacity in JIA.

[Therapeutic options in juvenile idiopathic arthritis : Surgical and conservative orthopedic rheumatological treatment]. / Therapeutische Optionen bei juveniler idiopathischer Arthritis : Operative und konservative orthopädisch-rheumatologische Behandlung.

The treatment of juvenile idiopathic arthritis (JIA) has made substantial progress within the last 25 years. Modern medicinal treatment enables inflammatory activity of the disease to be controlled in most of the cases. Mutilating courses of disease, which were formerly the rule have now become the exception. Today remission of disease is the aim of pediatric rheumatological treatment. Apart from effective control of inflammation this includes complete restoration of functional abilities of affected joints and the surrounding structures also affected. To achieve this goal a holistic and foresighted view of each patient's course is required. Therefore, even in an apparently uncomplicated course of disease in some cases of JIA it is advisable to plan an early interdisciplinary consultation including the pediatric rheumatologist and the orthopedic surgeon, in order to discuss an early surgical intervention, which can then be carried out in a timely manner, if necessary. This article provides an overview of the orthopedic rheumatological indications and options.

Molecular and Cellular Mechanisms of Arthritis in Children and Adults: New Perspectives on Applied Photobiomodulation.

Juvenile idiopathic arthritis and adult rheumatoid arthritis are two major groups with chronic joint pain and inflammation, extra-articular manifestations, and high risk of comorbidities, which can cause physical and ocular disability, as well as create great socio-economic pressure worldwide. The pathogenesis of arthritis manifested in childhood and adulthood is multifactorial, unclear, and overly complex, in which immunity plays an important role. Although there are more and more biological agents with different mechanisms of action for the treatment of arthritis, the results are not as expected, because there are partial responses or non-responsive patients to these compounds, high therapeutic costs, side effects, and so on; therefore, we must turn our attention to other therapeutic modalities. Updating knowledge on molecular and cellular mechanisms in the comparative pathogenesis of chronic arthritis in both children and adults is necessary in the early and correct approach to treatment. Photobiomodulation (PBM) represents a good option, offering cost-effective advantages over drug therapy, with a quicker, more positive response to treatment and no side effects. The successful management of PBM in arthritis is based on the clinician's ability to evaluate correctly the inflammatory status of the patient, to seek the optimal solution, to choose the best technology with the best physical parameters, and to select the mode of action to target very precisely the immune system and the molecular signaling pathways at the molecular level with the exact amount of quantum light energy in order to obtain the desired immune modulation and the remission of the disease. Light is a very powerful tool in medicine because it can simultaneously target many cascades of immune system activation in comparison with drugs, so PBM can perform very delicate tasks inside our cells to modulate cellular dysfunctions, helping to initiate self-organization phenomena and finally, healing the disease. Interdisciplinary teams should work diligently to meet these needs by also using single-cell imaging devices for multispectral laser photobiomodulation on immune cells.

Assessment and treatment of Down syndrome-associated arthritis: a survey of pediatric rheumatologists.

BACKGROUND: Inflammatory arthritis in children with Down syndrome (DS) was first described in 1984 and is now termed Down syndrome-associated arthritis (DA). Studies have shown that DA is under-recognized with a 19-month average delay in diagnosis. Additionally, most patients present with polyarticular, rheumatoid factor (RF) and anti-nuclear antibody (ANA) negative disease. Current therapies for juvenile idiopathic arthritis (JIA) have been used, but appear to be poorly tolerated, more toxic and less effective in patients with DA. There is currently no standardized approach to the assessment or management of DA. The objective of this study was to describe provider perspectives toward diagnostic and treatment approach of DA, to provide baseline information upon which to design future studies. METHODS: An electronic survey, organized into sections regarding individual practices of assessment and treatment approach of DA, was sent to the Pediatric Rheumatology electronic list-serv. Survey responses were voluntary and results were analyzed by descriptive statistics. RESULTS: Of 90 survey responses received, 89 were included in the analysis (one was a duplicate response). The respondents were mostly pediatric rheumatologist (94%), with greater than 10 years of experience (55%). The majority (64%) currently see 1-3 patients with DA. Most view DA as the same disease as JIA (73%), and the majority (63%) use a combination of history, exam and imaging to diagnose DA. The most ordered diagnostic tests are CBC (97%) and ESR (96%). The most used treatments include NSAIDs (94%) and methotrexate (91%) followed by anti-TNF agents (90%). Methotrexate is most administered by subcutaneous route (84%) at a dose of 15 mg/m2 (56%). Oral corticosteroids were only used in 19% of the patients with DA. CONCLUSION: This is the first study to evaluate provider perspectives towards the diagnostic and treatment approach of DA. Most pediatric rheumatologists feel that DA and JIA are synonymous, and similar approaches to diagnosis are employed, utilizing history, physical exam, laboratory tests, and imaging modalities. DA is treated similarly to JIA with initiation of NSAIDs, disease-modifying anti-rheumatic drugs and biologic therapy. More research is needed to determine optimal screening and therapeutic approach specific to DA.

Development and validation of the CAREGIVERS questionnaire: multi-assessing the impact of juvenile idiopathic arthritis on caregivers.

BACKGROUND: The primary caregiver is an important person in the life of patients with JIA. Their reactions depend on social, emotional and economic factors that affect the therapeutic alliance. Some generic instruments have been used to evaluate burden, anxiety, or quality of life of caregivers. This study aims to develop a specific instrument to measure the psychosocial and economic impacts on primary caregivers of patients with JIA. METHODOLOGY: This is a mixed methods research, that includes qualitative and quantitative data, and was carried out in two phases. First phase: a pragmatic qualitative study (questionnaire construction) was conducted in two parts, a non-systematic literature review followed by interviews with primary caregivers. Second phase: a cross-sectional study (questionnaire validation) to complete validation and estimate Cronbach's alphas based on tetrachoric correlation coefficients, correlation matrix and Cohen's kappa coefficient test. RESULTS: There were 38 articles found related to the experience of caregivers. 15 primary caregivers were interviewed (female 93%, median age 45 years). Thematic analysis identified 9 important topics from the perspective of participants (economic impact, coping, family roles, impact of diagnosis, mental health, couple/mate relationships, impact at work, religion, and knowledge of the disease). These topics were combined to create the interview questionnaire (56 items). Later, it was modified to 62 items that were divided into five dimensions: impact of the disease (psychosocial, economic, family, and relationships), knowledge of the disease, alternative medicine, future, and religion. The interview questionnaire was applied to 32 primary caregivers (female 93%, median age 37 years), results identify depression on 29 (90%), 18 (56%) feel sadness at diagnosis, 20 (63%) mentioned that JIA has influenced in their financial situation, 23 (72%) feel anxiety about the future, and 11 (37%) considered that their family relationships have changed. Statistical analysis identified inconsistencies during convergent and divergent validity of the construct. Consequently, 11 items were eliminated, 3 relocated, 6 modified, and 39 compacted obtaining the "Impact of Pediatric Rheumatic Diseases on Caregivers Multi-assessment Questionnaire" (CAREGIVERS questionnaire). This final version resulted on an eight-dimension (28 items) instrument. CONCLUSIONS: The CAREGIVERS questionnaire captures perspectives of both the participants and clinicians. It will be helpful to measure the impact of the disease and thus, to improve the quality of care of children with JIA and their families.

[Effectiveness of Watsu therapy in patients with juvenile idiopathic arthritis. A parallel, randomized, controlled and single-blind clinical trial]. / Efectividad de la terapia Watsu en pacientes con artritis idiopática juvenil. Un ensayo clínico controlado paralelo, aleatorio y simple ciego.

INTRODUCTION: Juvenile idiopathic arthritis (JIA) is a rheumatologic disease in children under 16 years old, which causes early physical disability. The use of hydrotherapy Watsu in these patients is propo sed. OBJECTIVE: To evaluate the effectiveness of Watsu compared to conventional hydrotherapy on health-related quality of life (HRQoL), functional health status, pain, and ranges of joint motion in patients with acute or subacute JIA. PATIENTS AND METHOD: Randomized (1:1) single-blind parallel controlled clinical trial in 46 patients with acute and subacute JIA between 8-18 years old. Pediatric Quality of Life Inventory 4.0 (PedsQL4.0), Childhood Health Assessment Questionnaire (CHAQ), and 10-joints Global range of motion score (GROMS) assessments were used at the beginning, post treatment, and after three months of follow-up. Patients were randomly assigned to the Watsu group (n = 24) and to the conventional hydrotherapy group (n = 22), participating in 10 sessions of 45 mi nutes once a week. RESULTS: Watsu therapy showed statistically significant improvements in physical functioning-HRQoL (p = 0.041), disability index (p = 0.015), distress index (p = 0.015), and functio nal health status-CHAQ (p = 0.013) after treatment compared to conventional hydrotherapy. Con clusions: Watsu therapy improved HRQoL, pain sensation, and functional health status compared to conventional hydrotherapy. Methodological adaptations are required in future studies to improve the external validity of these results.

Efectividad de la terapia Watsu en pacientes con artritis idiopática juvenil: un ensayo clínico controlado paralelo, aleatorio y simple ciego / Effectiveness of Watsu therapy in patients with juvenile idiopathic arthritis: a parallel, randomized, controlled and single-blind clinical trial

INTRODUCCIÓN: La artritis idiopática juvenil (AIJ), es una enfermedad reumatológica en menores de 16 años, que produce discapacidad física temprana. Se propone el uso de hidroterapia Watsu en estos pacientes. OBJETIVO: Evaluar la efectividad del Watsu en comparación a hidroterapia convencional en la calidad de vida relacionada con la salud (CVRS), estado de salud funcional, dolor y rangos articulares de movimiento en pacientes con AIJ aguda o subaguda. PACIENTES Y MÉTODO: Ensayo clínico controlado paralelo, con asignación 1:1, aleatorio y simple ciego, en 46 pacientes con AIJ en fase aguda y subaguda entre 8-18 años de edad. Se utilizaron las evaluaciones Pediatric Quality of Life Inventory 4.0 (PedsQL4.0), Childhood Health Assessment Questionnaire (CHAQ) y 10 joints-Global range of motion score (GROMS) al inicio, posterior al tratamiento y a los 3 meses de seguimiento. Los pacientes se asignaron aleatoriamente al grupo Watsu (n = 24) y al grupo hidroterapia convencional (n = 22), participando de 10 sesiones de 45 minutos, con frecuencia de una vez por semana. RESULTADOS: La terapia Watsu presentó mejoras estadísticamente significativas en funcionamiento ñsico-CVRS (p = 0,041), índice de discapacidad (p = 0,015), índice de malestar (p = 0,015) y estado de salud funcional-CHAQ (p = 0,013) posterior al tratamiento en comparación a la hidroterapia convencional. CONCLUSIONES: La terapia Watsu mejoró la CVRS, sensación de dolor y estado de salud funcional en comparación a la hidroterapia convencional. Se requiere de adaptaciones metodológicas en futuros estudios para mejorar la validez externa de estos resultados.

INTRODUCTION: Juvenile idiopathic arthritis (JIA) is a rheumatologic disease in children under 16 years old, which causes early physical disability. The use of hydrotherapy Watsu in these patients is proposed. OBJECTIVE: To evaluate the effectiveness of Watsu compared to conventional hydrotherapy on health-related quality of life (HRQoL), functional health status, pain, and ranges of joint motion in patients with acute or subacute JIA. PATIENTS AND METHOD: Randomized (1:1) single-blind parallel controlled clinical trial in 46 patients with acute and subacute JIA between 8-18 years old. Pediatric Quality of Life Inventory 4.0 (PedsQL4.0), Childhood Health Assessment Questionnaire (CHAQ), and 10-joints Global range of motion score (GROMS) assessments were used at the beginning, post treatment, and after three months of follow-up. Patients were randomly assigned to the Watsu group (n = 24) and to the conventional hydrotherapy group (n = 22), participating in 10 sessions of 45 mi nutes once a week. RESULTS: Watsu therapy showed statistically significant improvements in physical functioning-HRQoL (p = 0.041), disability index (p = 0.015), distress index (p = 0.015), and functio nal health status-CHAQ (p = 0.013) after treatment compared to conventional hydrotherapy. CONCLUSIONS: Watsu therapy improved HRQoL, pain sensation, and functional health status compared to conventional hydrotherapy. Methodological adaptations are required in future studies to improve the external validity of these results.

Therapeutic advances in juvenile idiopathic arthritis - associated uveitis.

PURPOSE OF REVIEW: The aim of this review is to highlight recent changes in the treatment of juvenile idiopathic arthritis (JIA) - associated uveitis in the era of biologics. RECENT FINDINGS: Early introduction of steroid-sparing therapies is paramount for appropriate management. Biologic therapies have improved the therapeutic management of JIA-uveitis and adalimumab is currently approved for pediatric-onset noninfectious chronic anterior uveitis with an inadequate response to topical steroids and methotrexate. Recent studies suggest that ocular complications in JIA-uveitis are less frequent compared with previous publications. However, patients with JIA-uveitis seem to be particularly dependent on classical immunosuppressive drugs or biologics. Indications for primary lens implantation have expanded considerably with the evolution of materials and better control of inflammation with biologics. The rate of serious adverse events related to new therapeutic approaches seem acceptable, however longer term follow-up is necessary. SUMMARY: Improvement in the initial screening and improved inflammation control with biologics has considerably reduced the potentially sight-threatening prognosis of JIA-uveitis.

Physical activity in patients with oligo- and polyarticular juvenile idiopathic arthritis diagnosed in the era of biologics: a controlled cross-sectional study.

BACKGROUND: Knowledge about objectively measured levels of physical activity (PA) and PA participation (included facilitators and barriers for PA) in patients with juvenile idiopathic arthritis (JIA) diagnosed in the era of biologics is limited. We aimed to compare objectively measured PA in patients with oligo- and polyarticular JIA diagnosed in the biologic era with controls and to examine associations between PA and disease variables; furthermore, to explore participation in PA, physical education (PE) and facilitators and barriers for PA participation in patients and controls. METHODS: The study cohort included 60 patients (30 persistent oligo JIA/30 poly-articular disease) and 60 age- and sex-matched controls. Age range was 10-16 years and 83% were female. PA was measured with accelerometry for seven consecutive days. Disease activity, current treatment, disease duration, functional ability, pain and fatigue were assessed. Structured interviews were applied to explore participation in PA and PE, and PA facilitators and barriers. RESULTS: Patients spent less time in daily vigorous PA than controls, (mean(SE) 21(2) min vs. 26(2) min, p = 0.02), while counts per minute (cpm), steps daily, sedentary time and light and moderate PA did not differ. No differences were found between JIA subgroups. The use of biologic medication was associated with higher cpm and lower sedentary time. Most patients and controls participated in organized or unorganized PA and PE, and enjoyment was the most reported facilitator for PA participation. More patients than controls reported pain as a PA barrier. CONCLUSION: The PA levels and participation in patients with oligo- and polyarticular JIA are mostly comparable to controls, but patients still need to be encouraged to increase vigorous PA. Enjoyment is the most important facilitator for PA participation in patients with JIA.

Contribution of the use of basic telemedicine tools to the care of children and adolescents with juvenile idiopathic arthritis at the Puerto Montt Hospital, Chile. / Aporte del uso de herramientas básicas de Telemedicina en la atención de niños y adolescentes con Artritis idiopática juvenil, en el Hospital de Puerto Montt. Chile.

Children and adolescents with rheumatologic diseases require specialized and comprehensive care, but pediatric rheumatologists and immunologists are concentrated in hospitals with specific, high-cost and modern technology. Considering that some patients with juvenile idiopathic arthritis (JIA) live in rural, remote and limited accessibility areas, the use of Telemedicine (TM) can optimize diag nosis, follow-up and prognosis. OBJECTIVE: Reporting 10 years of experience of a mixed care model: face-to-face and distance, using basic TM; the institutional impact, advantages, disadvantages and acceptance informed by parents and patients. PATIENTS AND METHOD: Exploratory, descriptive, and re trospective study with qualitative component. After the authorization of a scientific-ethics committee of the Reloncaví Health Service and the application of informed consent, a review of medical records was carried out and a qualitative survey was applied to parents and children over 14 years of age with JIA, seen between 2005-2015 in the pediatric ambulatory rheumatology polyclinic of Puerto Montt Hospital. RESULTS: The were 27/35 participating patients with JIA attended by a trained pediatrician and assisted by distance (1,000 km) by an immunologist. The 8/35 patients did not answer by choice or change of address. The 70% of parents and patients accepted the model of care and 4% would pre fer sporadic care only by specialists for diagnosis and follow-up. The number of patients transferred annually decreased from 10 to 1. The advantages of the care model outweighed the disadvantages perceived by parents and JIA patients. CONCLUSION: The use of TM tools in JIA decreased transfers, improved follow-up and were considered advantageous by patients and their parents.

Aporte del uso de herramientas básicas de Telemedicina en la atención de niños y adolescentes con Artritis idiopática juvenil, en el Hospital de Puerto Montt. Chile / Contribution of the use of basic telemedicine tools to the care of children and adolescents with juvenile idiopathic arthritis at the Puerto Montt Hospital, Chile

Resumen: Niños y adolescentes con enfermedades reumatológicas, requieren atención especializada e integral, sin embargo, reumatólogos e inmunólogos pediátricos se concentran en hospitales con tecnología específica, costosa y moderna. Como algunos pacientes con Artritis idiopática juvenil (AIJ) vive en áreas rurales, lejanas y de accesibilidad limitada, el uso de Telemedicina (TM) puede optimizar el diagnóstico, seguimiento y pronóstico. Objetivo: Mostrar 10 años de experiencia de un modelo de atención mixta: presencial y a distancia, usando TM básica; el impacto institucional, ventajas, des ventajas y aceptación reportados por padres y pacientes. Pacientes y Método: Estudio exploratorio, descriptivo, retrospectivo con componente cualitativo. Previa autorización de comité ético-científico del Servicio de salud del Reloncaví y la aplicación de consentimiento/asentimiento informado, se efectuó revisión de historias clínicas y se aplicó encuesta cualitativa a padres y niños mayores de 14 años con AIJ, atendidos entre 2005-2015 en el policlínico de reumatología infantil Hospital Puerto Montt. Resultados: Participaron 27/35 pacientes con AIJ atendidos por pediatra capacitado, aseso rado a distancia (1.000 km) por inmunólogo. 8/35 pacientes no contestaron por opción o cambio de domicilio. 70 % de padres y pacientes aceptaron el modelo de atención y 4% preferirían atención esporádica solo por especialista para diagnóstico y seguimiento. El número de pacientes trasladados anualmente disminuyó de 10 a 1. Las ventajas del modelo de atención superaron las desventajas per cibidas por padres y pacientes con AIJ. Conclusión: El uso de herramientas de TM en AIJ disminuyó los traslados, mejoró el seguimiento y fue considerado ventajoso por los padres y pacientes.

Abstract: Children and adolescents with rheumatologic diseases require specialized and comprehensive care, but pediatric rheumatologists and immunologists are concentrated in hospitals with specific, high-cost and modern technology. Considering that some patients with juvenile idiopathic arthritis (JIA) live in rural, remote and limited accessibility areas, the use of Telemedicine (TM) can optimize diag nosis, follow-up and prognosis. Objective: Reporting 10 years of experience of a mixed care model: face-to-face and distance, using basic TM; the institutional impact, advantages, disadvantages and acceptance informed by parents and patients. Patients and Method: Exploratory, descriptive, and re trospective study with qualitative component. After the authorization of a scientific-ethics committee of the Reloncaví Health Service and the application of informed consent, a review of medical records was carried out and a qualitative survey was applied to parents and children over 14 years of age with JIA, seen between 2005-2015 in the pediatric ambulatory rheumatology polyclinic of Puerto Montt Hospital. Results: The were 27/35 participating patients with JIA attended by a trained pediatrician and assisted by distance (1,000 km) by an immunologist. The 8/35 patients did not answer by choice or change of address. The 70% of parents and patients accepted the model of care and 4% would pre fer sporadic care only by specialists for diagnosis and follow-up. The number of patients transferred annually decreased from 10 to 1. The advantages of the care model outweighed the disadvantages perceived by parents and JIA patients. Conclusion: The use of TM tools in JIA decreased transfers, improved follow-up and were considered advantageous by patients and their parents.

Development of a national audit tool for juvenile idiopathic arthritis: a BSPAR project funded by the Health Care Quality Improvement Partnership.

Objective: Timely access to holistic multidisciplinary care is the core principle underpinning management of juvenile idiopathic arthritis (JIA). Data collected in national clinical audit programmes fundamentally aim to improve health outcomes of disease, ensuring clinical care is equitable, safe and patient-centred. The aim of this study was to develop a tool for national audit of JIA in the UK. Methods: A staged and consultative methodology was used across a broad group of relevant stakeholders to develop a national audit tool, with reference to pre-existing standards of care for JIA. The tool comprises key service delivery quality measures assessed against two aspects of impact, namely disease-related outcome measures and patient/carer reported outcome and experience measures. Results: Eleven service-related quality measures were identified, including those that map to current standards for commissioning of JIA clinical services in the UK. The three-variable Juvenile Arthritis Disease Activity Score and presence/absence of sacro-iliitis in patients with enthesitis-related arthritis were identified as the primary disease-related outcome measures, with presence/absence of uveitis a secondary outcome. Novel patient/carer reported outcomes and patient/carer reported experience measures were developed and face validity confirmed by relevant patient/carer groups. Conclusion: A tool for national audit of JIA has been developed with the aim of benchmarking current clinical practice and setting future standards and targets for improvement. Staged implementation of this national audit tool should facilitate investigation of variability in levels of care and drive quality improvement. This will require engagement from patients and carers, clinical teams and commissioners of JIA services.

A systematic review of psychosocial therapies for children with rheumatic diseases.

BACKGROUND: To assess the quality of evidence for the effects of psychosocial therapies on pain and function in children with rheumatic diseases. METHODS: We conducted a literature search of MEDLINE and PsycINFO for randomized clinical trials of psychosocial interventions for pain and disability in children with rheumatic diseases from January 1969 to September 2015. Studies with a sample size less than 10 subjects were excluded. Study quality was assessed using the Jadad score. RESULTS: Five articles met inclusion criteria, for a total of 229 patients, aged 5 to 18 years. Two studies included children with fibromyalgia. Three studies included children with juvenile arthritis. Neither study in fibromyalgia reported the statistical significance of immediate between-group pre-post changes in functioning or pain. One study examining the effects of an internet-based psychosocial intervention in children with juvenile arthritis reported significant differences in post-intervention pain scores (p = 0.03). However, 2 studies did not show improvements in pain scores among children with juvenile arthritis treated with psychosocial interventions vs. a wait-list control or vs. an active control (massage). No studies reported significant between-group differences for functional outcomes in children with juvenile arthritis. CONCLUSIONS: The available data were limited by the scarcity of randomized trials. Definite conclusions about the immediate effect of psychosocial interventions on pain and function in children with fibromyalgia could not be made because between-group comparisons of post-treatment change scores were not reported. For children with juvenile inflammatory arthritis, results of between-group comparisons for pain differed across studies, and analyses examining disability revealed no significant differences between groups.

Development and Preliminary Face and Content Validation of the "Which Health Approaches and Treatments Are You Using?" (WHAT) Questionnaires Assessing Complementary and Alternative Medicine Use in Pediatric Rheumatology.

OBJECTIVE: Complementary and alternative medicine (CAM) is commonly used by children with juvenile idiopathic arthritis (JIA), yet no validated questionnaires assess that use. The objective of this study was to develop child self- and parent proxy-report questionnaires assessing CAM use and to determine the face and content validity of the "Which Health Approaches and Treatments are you using?" (WHAT) questionnaires in pediatric rheumatology. METHODS: A sequential phased mixed methods approach was used to develop the questionnaires. A Delphi Survey of 126 experts followed by an interdisciplinary consensus conference of 14 stakeholders in CAM, general pediatrics and pediatric rheumatology was held to develop consensus on the content of the questionnaires using a nominal group technique. To determine face and content validity of the questionnaires, two groups, including (a) a purposive sample of 22 children with JIA 8 to 18 years and their parents from the Children's Hospital of Eastern Ontario and the Hospital for Sick Children, and (b) 21 Canadian pediatric rheumatology experts, participated in interviews. Participants were independently asked about the goal, understandability and comprehensiveness of the WHAT questionnaires, as well as the relevance of items. RESULTS: Consensus was reached on 17 items of the WHAT questionnaires. The domains found to be relevant were child's CAM use, factors associated with CAM use, perceived impact of CAM use, and communication about CAM. A total of 15 items in the parent proxy-report questionnaire and 13 items in the child report questionnaire showed adequate content validity. CONCLUSIONS: Consensus was reached by experts on the content of a pediatric CAM questionnaire. Face and content validity testing and modifications made to the WHAT questionnaires have helped ensure adequate preliminary validity for use in pediatric rheumatology. This constitutes the basis for further testing of these questionnaires in pediatric rheumatology and for adaptation to other chronic diseases.

Changes in fecal microbiota and metabolomics in a child with juvenile idiopathic arthritis (JIA) responding to two treatment periods with exclusive enteral nutrition (EEN).

The microbiome and immune system of the digestive tract are highly important in both health and disease. Exclusive enteral nutrition (EEN) is a common anti-inflammatory treatment in children with Crohn's disease in the European countries, and the mechanism is most likely linked to changes in the intestinal microbiome. In the present study, EEN was given in two treatment periods several months apart to a patient with very severe, disabling juvenile idiopathic arthritis (JIA), with a remarkable clinical response as the result. The aim of the present study was to study how the EEN treatment influenced the microbiome and metabolome of this patient. Fecal samples from before, during, and between treatments with EEN were studied. The microbiome was analyzed by sequencing of 16S rRNA amplicons using Illumina MiSeq, and the metabolome was analyzed using nuclear magnetic resonance. The microbiome changed markedly from treatment with EEN, with a strong reduction of the Bacteroidetes phylum. Metabolic profiles showed clear differences before, during, and between treatment with EEN, where butyrate, propionate, and acetate followed a cyclic pattern with the lowest levels at the end of each treatment period. This patient with JIA showed remarkable clinical improvement after EEN treatment, and we found corresponding changes in both the fecal microbiome and the metabolome. Further studies are needed to explore the pathophysiological role of the intestinal canal in children with JIA.

Impact of juvenile idiopathic arthritis on quality of life during transition period at the era of biotherapies.

OBJECTIVE: Few studies have assessed Health-Related Quality of Life (HR-QoL) in adults following juvenile idiopathic arthritis, and none since the advent of biotherapies. The aim of our study is to assess the impact of juvenile idiopathic arthritis on quality of life in a large transitional cohort, evaluate which factors influence quality of life in juvenile idiopathic arthritis, and determine which questionnaire should be used in practice. METHODS: All consecutive juvenile idiopathic arthritis patients followed during adulthood in a transitional care program were included. Demographical, clinical and biological data were collected. The following quality of life questionnaires were administered: SF36 and EuroQoL. Age- and sex-matched controls (without rheumatic disease) were included. RESULTS: One hundred and sixty-one juvenile idiopathic arthritis (120 women and 41 men) and 76 (51/25) controls were included. Out of 161, sixty-five (40%) were considered to be in remission. Juvenile idiopathic arthritis had a large impact on the physical scales of quality of life. Pain seemed to be the most important factor affecting quality of life in cases of juvenile idiopathic arthritis. No significant difference was found between sub-types of juvenile idiopathic arthritis. CONCLUSION: In this large transitional cohort of patients at the era of biotherapies, juvenile idiopathic arthritis has a larger effect on physical than mental scale of quality of life measures. Pain was the main factor influencing quality of life. Sub-types of juvenile idiopathic arthritis do not seem to influence quality of life.

Management of Children with Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) comprises a group of heterogeneous disorders of chronic arthritis in childhood and remains the commonest pediatric rheumatic disease associated with significant long-term morbidity. Advances in understanding of the pathogenesis, better definition of disease control/remission measures, and the arrival of biological agents have improved the outcomes remarkably. Methotrexate (Mtx) remains the first-line disease modifying (DMARD) therapy for most children with JIA due to its proven efficacy and safety. Sulphosalazine (SSz) (especially for enthesitis) and leflunomide may also have a secondary role. Tumor necrosis factor inhibitors (TNF-I), alone or in combination with Mtx have shown tremendous benefit in children with polyarticular JIA, enthesitis related arthritis (ERA) and psoriatic arthritis. Tocilizumab appears very efficacious in systemic arthritis and abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear, though clearly beneficial in adult RA. TNF-I with Mtx is also effective in uveitis associated with JIA. Biologicals have demonstrated an impressive safety record in children with JIA, although close monitoring for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of cancers is warranted.

Long-term safety and efficacy of varicella vaccination in children with juvenile idiopathic arthritis treated with biologic therapy.

OBJECTIVE: To evaluate the long-term safety and efficacy of varicella vaccination in children with juvenile idiopathic arthritis (JIA) treated with biologics. METHODS: We performed a prospective study with long term follow up. Six patients with JIA treated with biologics, received 2 doses of varicella vaccine. Before vaccination, JIA was stable on therapy and peripheral blood lymphocyte populations were within normal limits. After vaccination, children were followed for disease activity, infections and production of protective antibodies. RESULTS: There were no serious side effects after vaccination and no varicella infection. Disease activity remained stable. Five patients (83%) produced protective antibodies against varicella virus 6 weeks after the second vaccination. One patient with low level of protective antibodies got mild varicella infection 4 months after the second vaccination. CONCLUSION: Varicella vaccination appears to be safe in our group of six JIA patients treated with biologics. Vaccination does not always protect against varicella infection.