Improving care for children with juvenile idiopathic arthritis: the role of IL-6 inhibitors in a patient-centered approach

The medical management of juvenile idiopathic arthritis (JIA) and its complications has undergone significant changes in the last decade, a result largely of the introduction of biologics and increased availability of expertise in the diagnosis and management of rheumatic diseases in children and adolescents. The result is that clinical outcome has improved and complete disease control can often be achieved1. In 2010, the British Society for Paediatric and Adolescent Rheumatology (BSPAR) proposed guidelines for the optimal management of children and adolescents with JIA2. This advocacy statement emphasizes the importance of empowering children and their caregivers, facilitating early detection of JIA, prompt referral to a team of health professionals who are expert in the diagnosis and management of childhood rheumatic diseases, prompt access to all appropriate pharmacologic and biologic therapies, and regular followup and monitoring. The Canadian Wait Time Alliance sets acceptable wait times as 4 weeks in children with JIA, other than systemic onset JIA, and within 7 days of disease onset for children with systemic onset JIA. Screening for asymptomatic uveitis should take place within 4 weeks of the diagnosis of JIA3. Ideally, children and adolescents with JIA should be managed by a team of health professionals with training and experience in pediatric rheumatology given … Address correspondence to Dr. R.E. Petty, Division of Rheumatology, Department of Pediatrics, University of British Columbia, British Columbia’s Children’s Hospital, 4480 Oak St., Room K4-114, Vancouver, British Columbia V6H 3V4, Canada; E-mail: rpetty{at}cw.bc.ca

THU0498 The Patients' Experience of Imaging: Views from a Group Convened to Support the Development of Points to Consider for the Use of Imaging in the Diagnosis and Management of...

Juvenile idiopathic arthritis (JIA) is common in pediatric rheumatology. Despite treatment, many patients experience persistent disease activity. Joint hypermobility (JH), defined by an excessive range of motion across multiple joints, is prevalent in children and adolescents and may influence disease outcomes in JIA. This study examines the impact of JH on symptoms in youth and young adults with JIA. Data were obtained from the PR-COIN network and included patients under 21years old with a diagnosis of JIA. Patients with JIA and JH were matched with those having JIA-only based on age, sex assigned at birth, JIA subtype, and medication exposure. Clinical data, including disease activity measures, patient well-being, and pain ratings, were collected at baseline and follow-up visits. The sample included 420 patients with JIA + JH and 2100 with JIA only. The JIA + JH group exhibited higher disease activity at baseline, more active arthritis joints, elevated physician global assessment of disease activity scores, and worse patient-reported well-being. These differences persisted over time. The JIA + JH group had a 19-20% greater likelihood of maintaining high disease activity scores and worsening over subsequent visits, indicating a significant impact of JH on disease progression. JH in youth with JIA is associated with higher and persistent disease activity, suggesting that JH significantly contributes to the disease burden in patients with JIA and should be considered in treatment strategies. Future research should further explore the mechanisms by which JH influences disease activity and investigate comprehensive management approaches to improve outcomes for this population. Key Points • Children with JIA and joint hypermobility (JH) exhibit significantly higher disease activity at baseline compared to those with JIA only, including more active arthritis joints and elevated physician global assessment scores. • The presence of JH in JIA patients is associated with poorer patient-reported well-being and higher overall disease activity scores, which persist over time despite treatment. • JIA + JH patients have a 19-20% greater likelihood of maintaining high disease activity and worsening over subsequent visits, indicating a significant impact of JH on disease progression. • The study suggests that JH should be considered an important clinical factor in the management of JIA, with targeted interventions needed to address the increased disease activity and improve overall patient outcomes.

BackgroundThese updated guidelines aimed to provide appropriate and convenient guidelines for the treatment of various types of juvenile idiopathic arthritis (JIA).Using the Delphi technique, this study was conducted to reach expert consensus on a treat-to-target management strategy for JIA. According to the PICO (patient/population, intervention, comparison, and outcomes) approach, the preliminary scientific committee identified a total of 17 key clinical questions. To assemble evidence on the advantages and dangers associated with JIA treatments, an evidence-based, systematic literature review was conducted. Researchers and clinicians with experience in JIA management were identified by the core leadership team. To establish a consensus on the management suggestions for JIA patients, a Delphi approach (2 rounds) was used.ResultsAn online survey was applied to the expert panel (n = 27), and 26 of them completed both rounds. At the conclusion of round 2, a total of eighteen (18) recommendation items were gathered, which were divided into four sections to address the four key JIA categories. The percentage of those who agreed with the recommendations (ranks 7–9) ranged from 83.2 to 100% (average 86.8%). The phrasing of all 18 clinical standards identified by the scientific committee was agreed upon (i.e. 75% of respondents strongly agreed or agreed). Algorithms have been proposed for the management of JIA polyarthritis, oligoarthritis, and systemic JIA.ConclusionA wide and representative panel of experts initiated a consensus about the management of JIA. The created guidelines give a complete approach to the management of JIA for all healthcare professionals involved in its management, as well as a means of monitoring and evaluating these guidelines on a regular basis.

Purpose of ReviewThis 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 FindingsDespite 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.SummaryGlobally, 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.

Juvenile idiopathic arthritis (JIA) is the most common chronic joint disease in paediatric rheumatology. Over the last two decades, ultrasound (US) has emerged as a tool with the potential to enhance disease assessment and management of JIA. This imaging modality is safe and well tolerated by children and can be easily applied bedside in the clinical setting. Owing to the lack of published studies regarding the validity and reproducibility of US in JIA and the difficulties in interpreting images of children, US was initially perceived like an art rather than a science. In recent years, a great deal of efforts has been made in order to fill the gap of scientific knowledge on US between paediatric and adult rheumatology. This has yielded significant breakthroughs, such as the achievement of valuable information about the anatomical peculiarities of the growing skeleton on US, including internationally agreed definitions on B-mode and Doppler US of components for the normal joints, and the development of a standardised scanning protocol for US examination suitable for use in children. The precise role of US in JIA, however, is yet to be fully defined. Although further research regarding the use of US in joint inflammatory pathology in paediatrics is required, this imaging modality may well possess the necessary properties to pursue the best practice in the care of children with JIA in the near future. The present review provides information on the recent advances that have made the application of US increasingly promising for the management of JIA.

ObjectivesAlthough the management of juvenile idiopathic arthritis (JIA) is well-established in the pediatric context, management in adulthood and long-term outcomes, including mental health concerns, are less understood, limited in part by reclassification to adult diagnoses, such as rheumatoid arthritis (RA) and ankylosing spondylitis (AS). Current management of adults with JIA is largely extrapolated from pediatric strategies; it is unclear if this is best practice. This study’s objective is to discern patterns of care and natural history of JIA after patients enter Adult Rheumatology care.MethodsPatients with JIA followed by Adult Rheumatology at a large Edmonton site were identified through the electronic medical record. Patients were excluded if they were under age 17 or their diagnosis was reclassified prior to age 18. A retrospective chart review was completed to collect information including the transition into Adult Rheumatology care, current disease status and classification, DMARD use, uveitis frequency and monitoring, and mental health interventions.Results181 patients were identified (135 female). Most patients (74.3%) were referred from Pediatric Rheumatology while 5.4% were referred from elsewhere but were previously followed by Pediatric Rheumatology; 20% did not have transfer-of-care details available. 74% were in remission or low disease activity state, of whom 17.9% were in drug-free remission when transitioned to adult care. 41.9% were reclassified under a new diagnosis in adulthood: RA (26.2%), psoriatic arthritis (5.8%), AS (7.6%), and IBD-associated inflammatory arthritis (2.3%). 40.6% had documented uveitis screening in adulthood; 79.3% with prior childhood uveitis had ongoing ophthalmologic surveillance. All patients diagnosed with uveitis in adulthood had uveitis in childhood; 41.1% of children with uveitis experienced recurrence in adulthood. As children, 28.2% sought mental health services. Further, 35.4% were treated for mental illness in adulthood, while 4.4% received inpatient psychiatric treatment as a child or adult.ConclusionOur study identified a cohort of patients with JIA followed by Adult Rheumatology; nearly half were reclassified to an alternate Adult Rheumatology diagnosis, which may contribute to the challenges of managing and monitoring long-term outcomes of patients with JIA. Purposeful ongoing uveitis surveillance in adulthood was fair (79%) and captured all patients who experienced uveitis in adulthood, highlighting its importance in this particular group. The high prevalence of mental illness treatment within this cohort suggests that patients with JIA may benefit from monitoring for mental health issues.Best Abstract by a Medical Student Award

French protocol for the diagnosis and management of juvenile idiopathic arthritis including pediatric-onset Still's disease.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and causes short- and long-term disability. Optimal management requires pharmacologic and non-pharmacologic interventions. Few studies have explored the youth and family experience of the management of JIA. This study's objective was to explore the management experience of youth with JIA and their parents. This qualitative study used semi-structured interviews with youth 12-18years of age with JIA receiving biological medication and parents of children with JIA on biological medication. Participants were recruited in clinics using convenience sampling. A thematic analysis approach was employed for data analysis. Nine youth and 14 parents participated. Four themes were identified that encompassed an overarching theme of participants managing JIA within the context of their life: aspects of life affected by JIA and its management, lived experience with JIA management, medication decision-making, and involvement in decision-making. Juvenile idiopathic arthritis management is situated within the context of their life but is normally (outside acute events) not central. Two dimensions were added to those in the literature: parents' overall approaches to health and the sense of urgency surrounding decision-making. Our findings reinforce the importance of person- and family-centred care in paediatric rheumatology. That is, identifying what matters most to youth and their parents given their current life circumstances to provide a foundation for discussions of how they want to manage their JIA.

The evidence base that underlies the management of children and young people with paediatric rheumatic diseases is deficient. In this field, there are many crucial unanswered questions. The UK Paediatric Rheumatology Clinical Studies Group, supported by UK National Institute for Health Research Clinical Research Network: children and Versus Arthritis, elicited ideas for research priorities from paediatric rheumatologists, trainees, allied health-care professionals, nurse specialists, patients, parents of patients, carers, and charities. These ideas were collected through online surveys and face-to-face meetings. A modified Delphi process was used, which included online research priority ranking surveys and a consensus workshop. A longlist of 55 disease-specific research priorities and 37 general research priorities were voted on in the first survey. A list of 11 top general research priorities was produced. The top ten disease-specific research priorities were discussed in depth at a Delphi workshop to determine their final ranking. This Health Policy paper will help to guide clinicians, academics, and funding bodies to prioritise research in paediatric rheumatic diseases, specifically in areas of unmet patient needs.

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritis of childhood, characterized by various clinical phenotypes associated with variable prognosis. Significant progress has been achieved with the use of biologic treatments, which specifically block pro-inflammatory molecules involved in the disease pathogenesis. The most commonly used biologics in JIA are monoclonal antibodies and recombinant proteins targeting interleukins 1 (IL-1) and 6 (IL-6), and tumor necrosis factor α (TNF-α). Several biomarkers have been investigated in JIA.Aims: To assess the level of evidence available regarding the role of biomarkers in JIA related to guiding clinical and therapeutic decisions, providing disease prognostic information, facilitating disease activity monitoring and assessing biologic treatment response in JIA, as well as propose new strategies for biologic therapy-related biomarker use in JIA.Methods: We searched PubMed for relevant literature using predefined key words corresponding to several categories of biomarkers to assess their role in predicting and assessing biologic treatment response and clinical remission in JIA.Results: We reviewed serological, cellular, genetic, transcriptomic and imaging biomarkers, to identify candidates that are both well-established and widely used, as well as newly investigated in JIA on biologic therapy. We evaluated their role in management of JIA as well as identified the unmet needs for new biomarker discovery and better clinical applications.Conclusion: Although there are no ideal biomarkers in JIA, we identified serological biomarkers with potential clinical utility. We propose strategies of combining biomarkers of response to biologics in JIA, as well as routine implementation of clinically acceptable imaging biomarkers for improved disease assessment performance.

AB1003 ANTI-DFS70 AUTOANTIBODIES, JUVENILE IDIOPATHIC ARTHRITIS (JIA) AND UVEITIS: ARE ANTI-DFS70 A PREDICTIVE MARKER OF UVEITIS RISK IN JIA PATIENTS?

The management of juvenile idiopathic arthritis (JIA) is based on a combination of pharmacological interventions, physical and occupational therapy, and psychosocial support. Ideally, the management is conducted by a multidisciplinary team composed by a paediatric rheumatologist, specialist nurse, physical therapist, occupational therapist, and psychologist. The treatment is aimed to achieve disease control, to relieve pain, to foster normal nutrition and growth, to maintain physical and psychological well-being, and to prevent long-term damage related to the disease or its therapy. First-line pharmacological interventions are based on non-steroidal anti-inflammatory drugs and intra-articular corticosteroids. Patients who are refractory to these therapies are candidates to receive disease-modifying anti-rheumatic medications, namely methotrexate or, in case of enthesitis-related arthritis, sulfasalazine. If therapeutic response is inadequate or suboptimal, the introduction of a biologic response modifier is considered. Systemic corticosteroids are used in selected instances, which include the management of extra-articular manifestations of systemic arthritis or the achievement of quick disease control while are awaiting the full therapeutic effect of a disease-modifying agent in patients with severe polyarthritis. To help physician select the safest and most effective treatment for JIA, the American College of Rheumatology (ACR) has issued a set of recommendations that were meant to be as evidence based as possible. The British Society for Paediatric and Adolescent Rheumatology (BSPAR) has developed the standards of care for patients with JIA, which are aimed to help the paediatric rheumatology teams to improve the service they provide.

FRI0503 Eular-Pres Points to Consider for the Use of Imaging in the Diagnosis and Management of Juvenile Idiopathic Arthritis in Clinical Practice

THU0525 DEVELOPMENT OF A PREDICTIVE TOOL FOR RESPONSE TO ANTI-TNF-ALPHA THERAPY IN JIA USING GENE EXPRESSION PROFILES IN PERIPHERAL DERIVED MONONUCLEAR CELLS