Abstract
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.
Summary
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.
Similar content being viewed by others
Avoid common mistakes on your manuscript.
Background
Evolution of Juvenile Idiopathic Management
Paradox of the Paediatric Orphan
Pharmaceutical companies have, for a long time, shunned paediatric research by ignoring the importance of therapeutic trials of medication in children [1,2,3,4]. This was because pharmaceutical companies considered the paediatric population as a small and unlucrative market to sponsor, where their cost of rigorous clinical trials could not be reclaimed [5, 6]. There were also ethical issues related to the recruitment of children [1, 7], paediatric dosing, and the stress children experience with blood sampling [2].
Instead, pharmacological testing disproportionally represented adults [1, 4, 8]. In order to evade paediatric endorsement, pharmaceutical companies placed a clause on medication labels, not recommending their medications for paediatric usage [7, 9]. In fact, in 1991 in the USA, 81% of medications contained this disclaimer [10], and as a consequence, most medications prescribed for children were officially not licensed (unlicensed); or used outside of the terms of the license (off-labelled) [2,3,4]. This meant prescribers were left to draw on the results of adult studies to conclude the child’s therapeutic response and dosage requirements [1, 4], whilst making assumptions as to the physical and physiological differences between adults and children [7, 10], and the pharmacokinetic variances seen from childhood to young adulthood (neonates, infants, children, and adolescents) that can alter drug absorption, distribution, metabolism and excretion [4, 11]. As a result, most medications (67 to 91%) prescribed to children had no efficacy, safety or quality testing [2, 4, 12], potentially exposing children to ineffective treatments, medication toxicity or other serious adverse events [13, 14].
Legislative Changes
Fortunately, over the last two decades, legislative changes came into place, boosting paediatric drug trials [7], and improving paediatric labelling [4, 15]. In the USA for example, the Best Pharmaceuticals for Children Act (2002) identified a priority list of medications that needed further study [7]. Whilst the Pediatric Research Equity Act (2003) required all new medications to include a paediatric assessment, unless waived [7, 15], within 5 years, information on efficacy and safety had been added to more than 400 medications, for neonates, infants, children, and adolescents [7, 15]; and these changes were often surprising. For Juvenile Idiopathic Arthritis (JIA), this brought about an increase in the dosage requirements (per-kilogram) for the commonly prescribed nonsteroidal anti-inflammatory drug (NSAID) Etodolac (now discontinued). Children aged 6 to 16 years required twice the lower dose recommended for adults [8]. Similar changes spread, for example in Australia (2014), the Therapeutics Goods Administrator adopted the European Medicine Agency Guidelines on Pharmaceutical Development, to ensure safe data generation and quality paediatric medications [16].
Rare and Ignored
Another unprofitable market ignored by pharmaceutical companies was the treatments for rare diseases [5]. In response, in the USA, the Orphan Drug Act (1983) was passed, aiming to stimulate investments by providing financial incentives to reduce research and marketing costs. Similar policies have been followed in Japan (1993), Australia (1997) and Europe (1999) [17], to promote pharmaceutical development in small populations [18, 19]. For JIA, these legislative changes improved the availability of treatments targeting specific subtypes [20].
Treatment Guided by Expert Opinion and Close Monitoring
Due to a lack of paediatric specific research, the treatment of JIA was conservatively based on adult rheumatic disease [21]. Adult mainstay treatment followed the principles of the therapeutic pyramid [22], in other words, an escalation of steps [23], to target excessive inflammation and pain [3, 21]. Generally, this was achieved through mild analgesia, NSAIDs such as salicylic acid (Aspirin), and if needed, corticosteroids [3, 21, 24]. Children were then closely monitored for side effects, such as gastrointestinal irritation and hepatotoxicity [24], osteopenia and growth retardation [25]. In addition, physiotherapy and splinting were keystone in preserving functional ability and preventing contracture [21]. Clinically, less than half of the children gained adequate control of their disease [26, 27], and remained at risk of chronic pain and disease-related disability [28]. Psychological support was also provided to the family by treating physicians, nurses, and if needed, a social worker [21].
Early Aggressive Treatment and the Promotion of Self-Management
Fortunately, in the 1980s, a major breakthrough occurred in rheumatology. A small window of opportunity to suppress the disease was identified in adult research studying early arthritis [29]. This turned the principles guiding the therapeutic triangle upside down [30], as the focus of care turned to early aggressive treatment to control disease and prevent tissue damage [23, 29]. The disease-modifying antirheumatic drugs (DMARD), methotrexate (MTX), became the forefront of treatment, for children that failed to respond sufficiently to the NSAIDs [3, 31]. MTX is a folic acid analogue that inhibits the folate pathway, interfering with tissue cell reproduction [32], transforming the outlook for many children with JIA [29]. The use of NSAIDs and corticosteroids moved to become an adjunctive treatment [28]. During this MTX era, a strong focus was also placed on educating children and their parents to promote medication adherence to optimise efficacy; and to monitor for side effects, because of the safety issues associated with MTX, for example increased liver enzymes [29].
A New Focus: The Pathogenesis of JIA
Through advancements in molecular medicine, a greater understanding was developed on inflammatory arthritides and cytokine networks [33]. This resulted in therapeutic treatments focusing on the pathogenesis of JIA, specifically the proinflammatory cytokines such as tumour necrosis factor alpha [TNFα], interleukin-1 [IL-1], and interleukin-6 [IL-6]; and the signalling mediators that regulated the B-cell and T-cell lymphocytes [34]. This major breakthrough is known as the biological era [35], increasing the number and types of biological DMARD treatments, that are based on varying modes of action, for example, Etanercept (TNFα antagonist) [32], Anakinra (IL-1 antagonist) and Tocilizumab (IL-6 inhibitor) to block the inflammatory process [34]. This brought about new treatments for individual subtypes.
Importantly, recently published cohort studies are now recording advancements in clinical care, post the biological era. Including, for many children, inactive disease (range 40–50%) [35], and disease remission (range 33 to 47%) [36••, 37•]; resulting in a marked reduction in disease damage [35]. However, there is still a substantial proportion of children whose course of disease could further be improved [35]. The need for close monitoring is also still needed, due to the antagonistic affects these medications can have on immunological pathways, placing children at an increased risk of infection [38].
Statement of Current Benchmark
The current benchmark for the management of JIA recommended by the NSW Agency for Clinical Innovation [39], the British Society for Paediatric and Adolescent Rheumatology [40], the American College of Rheumatology [41, 42], and the Canadian Rheumatology Association [43] encourages the following: early referral to health professionals specialising in paediatric rheumatic diseases, to support early diagnosis and the initiation of treatment. This treatment, then needs to be frequently assessed and adjusted accordingly, to achieve inactive disease and no pain [43].
Specialised Paediatric Rheumatology Centres
Further, to achieve the best possible health outcome, the management of JIA requires the support from a diverse interdisciplinary team, over a long period of time [44]. The forefront of this support is provided through specialised Paediatric Rheumatology (PR) Centres, typically based in tertiary children’s hospitals in major capital cities. These centres allow children and parents access to a one stop shop that delivers a range of holistic support and treatments that target both rheumatological and paediatric specific needs [39, 45, 46]. This is important because there are major differences between adult and paediatric care [39]. For example, physiotherapists may provide guidance on school physical education, sporting and home programs [46]. Nursing may recommend supportive strategies to parents for their child and their siblings [40] or provide practical advice on how to administer medications to children [40, 46], whilst podiatry monitors foot health and motor milestone development [47, 48], and ophthalmology screens eye health, in children too young to report symptoms [49•]. A crucially essential interdisciplinary care model needed to be available to all.
Global Challenges Hindering Paediatric Rheumatology
Internationally, the current demand on PR services far exceeds supply because of chronic shortages in the PR workforce [39, 50,51,52]. In fact, in Australia in 2021, there were only 20 PRs instead of the 61 recommended to meet the current workload. This means there is a 67% deficit, based on the minimum standards of care [53]. Globally, this workforce shortage shadows at 88%, with Asia and Africa being the worst affected locations [51, 54], despite their large paediatric populations [54]. Africa, for example, has only 10 PR centres across the 54 countries [55]; and China, 50 centres, even after three decades of pursuing improvements [56]. Also, in most countries, this workforce shortage is exacerbated in regional and rural areas [39]. Therefore, the standard of PR care falls below the expected benchmark [39], and this workforce shortage is expected to become worse [50], suggesting a serious problem and the delivery of suboptimal care [39, 57]. This will place children at risk of delayed and inadequate treatment, and poor outcomes from their disease.
One main reason has been the slow recognition of PR as a subspecialty [58,59,60]. As a result, medical schools have provided minimal paediatric education on musculoskeletal and rheumatic diseases [60], which has limited PR exposure and the uptake of residency programs offering PR rotations [61]. Consequently, this has had an impact on recruitment into the PR workforce, and a lack of trained staff to teach, and conduct research [52, 60, 62, 63]. This lack of knowledge then transcends across the disciplines, for example in orthopaedics and by general paediatricians [60, 64, 65]. It is not then surprising that those healthcare practitioners who are at the first point of contact with the healthcare system: general practitioners, emergency room physicians and school nurses, often overlook a child’s joint swelling and restriction [65, 66]. This results in diagnostic errors and delayed referral to the specialised PR centres, hindering a correct diagnosis and the provision of appropriate treatment [65, 67]. Concerningly, a cohort study of 130 PR centres across 49 countries, identified that these diagnostic delays were further intensified in low Gross Domestic Profit (GDP) countries [68••]. Inevitable, considering low GDP countries are still being inundated with communicable diseases, and their health care resources are limited. Further hindering their ability to apply the current standards of care [54, 69,70,71]; resulting in higher rates of disease activity, and more disease damage [68••].
Recently in Australia, the National Action Plan for the Health of Children and Young People 2020–2030, has put forth the importance of raising awareness of rare diseases, to enhance support, and improve health promotion, to allow children to thrive [72]. In addition, there is a call for new innovative approaches to address the current health inequalities, and indeed, better equipped the healthcare workforce in identifying and addressing a child’s healthcare needs [72]. Therefore, it is imperative that the way forward in JIA management is to understand the current barriers that need to be addressed in the management of JIA, to finally improve the health care disparities in access, treatment, and outcomes for children living with JIA.
We propose, a resource-efficient way to improve the level of care provided could be achieved by embedding digital health into clinical practice, through the use of digital sharing technologies [73]. For example, to create an integrative care model between the children, general practice and the PR team. To improve fragmented service delivery and the coordination of interdisciplinary care, across the healthcare system [74]. Also, essentially, allowing for ongoing monitoring and timely support to children with JIA, when needed.
Rise in Telehealth Consultations
Understandably, the COVID-19 pandemic generated a rise in telehealth in PR. Despite the criticism, because physical examinations are needed, Paediatric Rheumatologists confirm they were still able to verify diagnosis and treatment responses by using repeated visualization and feedback from parents [75]. However, several studies report, the assessments which commonly support treat-to-target decisions were not fully utilised, when compared to face-to-face consultations [76, 77]. Therefore, improvements are needed in the delivery of the virtual examination. Foremost, current assessment tools need to be validated [77], or adapted [78•], to ensure ease of use, and their virtual effectiveness [77]. As seen, for example, with the Video-pGALS (video paediatric Gait Arms Legs Spine) [78•], or eVAS (electronic visual analogue scale) [79].
New Emerging Models of Digital Healthcare
In the past decade, there have been profound changes in the types of digital technologies that have become available to support chronic disease management [80]. Mobile digital devices such as smartphones, tablets and smart watches can now connect to the Internet from almost any location [81, 82]; and are capable of reliably monitoring and assessing in real-time, crucial health indicators that could be used to support successful PR management.
For JIA, the benefits of remote monitoring are emerging [83]. For example, Stinson et al. reported that real-time pain assessments in electronic pain diaries, reduced recall bias [84]. Whilst Doeleman et al. demonstrated that quality of life questionnaires assessed through an app, asserted disease activity [85•]. Further, completing web-based assessments, before the Paediatric Rheumatologist consultation improved time efficiency [84] and promoted psychosocial conversation [86]. In addition, longitudinal monitoring of pain, medication adherence, and physical activity can be viewed on a web-based platform, captured through smart watch and mobile app technology [87]. However, most of this research is still at a very early stage of evaluation [83, 87]. Nonetheless, it is still laying the foundations to a new model of healthcare delivery. Notably, this model of digital health care also asserts the principles of person-centred care, because it requires engagement from children [84, 85•, 86, 87], and has the potential to improve communication between the child, parent and their wider healthcare team [88].
Therefore, it is crucial that future research and advancements in 5G digital health interventions should directly focus on addressing the long-standing health inequalities experienced by children with JIA, particularly for those living in remote or regional areas; in fact, any area or country far from Paediatric Rheumatologist and specialised PR centres.
Conclusion
The significance of this critical review is to provide an overview of JIA management, by highlighting the journey of the paediatric orphan through to the current benchmark of best practice, in order to identify the long-standing limitations that have hindered the development of successful JIA management. In addition, a strong emphasis was placed on the need to frequently assess and modify JIA-related treatments accordingly, to achieve inactive disease and no pain. Yet meeting this current benchmark is difficult. The demand for services far exceeds service delivery, due to the global PR workforce shortage, resulting in, for many children, suboptimal care. Raising awareness of these issues is the first step in reducing these current health inequalities, to ensure health care organisations and policy makers understand the need, to increase, and better equip the PR interdisciplinary workforce through using digital health technology.
Data Availability
The data that support the findings of this study is available on request from the corresponding author, sonia.butler@newcastle.edu.au.
References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Gazarian M. Why are children still therapeutic orphans? Aust Prescr. 2003;26(6):122–3. https://doi.org/10.18773/austprescr.2003.090.
Conroy S, McIntyre J, Choonara I, Stephenson T. Drug trials in children: problems and the way forward. Br J Clin Pharmacol. 2000;49(2):93–7. https://doi.org/10.1046/j.1365-2125.2000.00125.x.
Becker ML. Role of methotrexate in juvenile idiopathic arthritis: where we have been and where we are going. Int J Clin Rheumatol. 2013;8(1):123–135. https://www.openaccessjournals.com/articles/role-of-methotrexate-in-juvenile-idiopathic-arthritis-where-we-have-been-and-where-we-are-going.pdf Accessed 4 May 2023.
Roberts R, Rodriguez W, Murphy D, Crescenzi T. Pediatric drug labeling improving the safety and efficacy of pediatric therapies. JAMA. 2003;290(7):905–11. https://doi.org/10.1001/jama.290.7.905.
Phillips MI. Big Pharma’s new model in orphan drugs and rare diseases. Expert Opin Orphan Drugs. 2013;1(1):1–3. https://doi.org/10.1517/21678707.2013.752128.
Wilson JT. An update on the therapeutic orphan. Pediatrics. 1999;104(3 Pt 2):585–90. https://doi.org/10.1542/peds.104.S3.585.
Burckart GJ, Kim C. The revolution in pediatric drug development and drug use: therapeutic orphans no more. J Pediatr Pharmacol Ther. 2020;25(7):565–73. https://doi.org/10.5863/1551-6776-25.7.565.
Institute of Medicine. Addressing the barriers to pediatric drug development: workshop summary. Washington, DC: The National Academies Press; 2008. p. 1–64. https://doi.org/10.17226/11911.
Shirkey H. Editorial comment: Therapeutic orphans. J Pediatr. 1968;72(1):119–20. https://doi.org/10.1016/S0022-3476(68)80414-7.
Gilman JT, Gal P. Pharmacokinetic and pharmacodynamic data collection in children and neonates A quiet frontier. Clin Pharmacokinet. 1992;23(1):1–9. https://doi.org/10.2165/00003088-199223010-00001.
O’Hara K. Paediatric pharmacokinetics and drug doses. Aust Prescr. 2016;39:208–10. https://doi.org/10.18773/austprescr.2016.071.
Lindell-Osuagwu L, Korhonen MJ, Saano S, Helin-Tanninen M, Naaranlahti T, Kokki H. Off-label and unlicensed drug prescribing in three pediatric wards in Finland and review of the international literature. J Clin Pharm Ther. 2009;34:277–87. https://doi.org/10.1111/j.1365-2710.2008.01005.x.
Bellis JR, Kirkham JJ, Nunn AJ, Pirmohamed M. Adverse drug reactions and off-label and unlicensed medicines in children: a prospective cohort study of unplanned admissions to a paediatric hospital. Br J Clin Pharmacol. 2014;77(3):545–53. https://doi.org/10.1111/bcp.12222.
Turner S, Nunn A, Fielding K, Choonara I. Adverse drug reactions to unlicensed and off-label drugs on paediatric wards: a prospective study. Acta Paediatr. 1999;88(9):965–8. https://doi.org/10.1080/08035259950168469.
Christensen M. Best Pharmaceuticals for Children Act and Pediatric Research Equity Act: time for permanent status. J Pediatr Pharmacol Ther. 2012;17(2):140–1. https://doi.org/10.5863/1551-6776-17.2.140.
Thearputics Goods Administration. International scientific guideline: guideline on pharmaceutical development of medicines for paediatric use; 2014. https://www.tga.gov.au/resources/resource/international-scientific-guidelines/international-scientific-guideline-guideline-pharmaceutical-development-medicines-paediatric-use Accessed 4 May 2023.
Orphanet. The portal for rare diseases and orphan drugs: about orphan drugs; 2023. https://www.orpha.net/consor/cgi-bin/Education_AboutOrphanDrugs.php?lng=EN Accessed 2 May 2023.
Therapeutic Goods Administration. Orphan drugs program: discussion paper, May 2015. Commonwealth of Australia: ACT 2015. https://www.tga.gov.au/sites/default/files/consultation-orphan-drugs-program.pdf Accessed 1 May 2023.
European Medicine Agency. Paediatric medicines: Overview. 2021. https://www.ema.europa.eu/en/human-regulatory/overview/paediatric-medicines-overview Accessed 4 May 2023.
Lutz T, Lampert A, Hoffmann GF, Ries M. Novel treatments for rare rheumatologic disorders: analysis of the impact of 30 years of the US orphan drug act. Orphanet J Rare Dis. 2016;11(1):60–60. https://doi.org/10.1186/s13023-016-0443-x.
Ansell BM, Swann M. The management of chronic arthritis of children. J Bone Joint Surg Br. 1983;65(5):536–43. https://doi.org/10.1302/0301-620X.65B5.6643554.
Wilske KR. Remodeling the therapeutic pyramid: evolving therapeutic strategies for rheumatoid arthritis. Japan J Rheumatol. 1999;9(1):1–16. https://doi.org/10.3109/BF03041255.
Hinze C, Gohar F, Foell D. Management of juvenile idiopathic arthritis: hitting the target. Nat Rev Rheumatol. 2015;11(5):290–300. https://doi.org/10.1038/nrrheum.2014.212.
Baum J. Aspirin in the treatment of juvenile arthritis. Am J Med. 1983;74(6A):10–5. https://doi.org/10.1016/0002-9343(83)90523-5.
Saggese G, Vierucci, F. Systemic corticosteroids for inflammatory disorders in pediatrics. In: C Roland, ed. Systemic corticosteroids for autoimmune/inflammatory disorders in children: introduction. Switzerland: Adis, Springer International Publishing; 2015.
Minden K, Kiessling U, Listing J, et al. Prognosis of patients with juvenile chronic arthritis and juvenile spondyloarthropathy. J Rheumatol. 2000;27(9):2256–63.
Minden K. Adult outcomes of patients with juvenile idiopathic arthritis. Horm Res. 2009;72(1):20–5. https://doi.org/10.1159/000229759.
Stoll ML, Cron RQ. Treatment of juvenile idiopathic arthritis: a revolution in care. Pediatr Rheumatol Online J. 2014;12:13–13. https://doi.org/10.1186/1546-0096-12-13.
Ramanan AV, Whitworth P, Baildam EM. Use of methotrexate in juvenile idiopathic arthritis. Arch Dis Child. 2003;88(3):197. https://doi.org/10.1136/adc.88.3.197.
Levinson JE, Wallace CA. Dismantling the pyramid. J Rheumatol Suppl. 1992;33:6–10.
Truckenbrodt H, Häfner R. Methotrexate therapy in juvenile rheumatoid arthritis: a retrospective study. Arthritis Rheum. 1986;29(6):801–7. https://doi.org/10.1002/art.1780290616.
MIMS online. Pfizer (Australia) Methotrexate Injection BP. 2021. https://www.mimsonline.com.au Accessed 2 March 2 2023.
Spiegel L, Kristensen KD, Herlin T. Juvenile idiopathic arthritis characteristics: etiology and pathophysiology. Sem Orthod. 2015;21(2):77–83. https://doi.org/10.1053/j.sodo.2015.02.003.
Hayward K, Wallace CA. Recent developments in anti-rheumatic drugs in pediatrics: treatment of juvenile idiopathic arthritis. Arthritis Res Ther. 2009;11(1):216. https://doi.org/10.1186/ar2619.
Giancane G, Muratore V, Marzetti V, et al. Disease activity and damage in juvenile idiopathic arthritis: methotrexate era versus biologic era. Arthritis Res Ther. 2019;21(1):168–168. https://doi.org/10.1186/s13075-019-1950-7.
•• Glerup M, Rypdal V, Arnstad ED, et al. Long-term outcomes in juvenile idiopathic arthritis: eighteen years of follow-up in the population-based Nordic juvenile idiopathic arthritis cohort. Arthritis Care Res (Hoboken). 2020;72(4):507–16. https://doi.org/10.1002/acr.23853. This 18-year cohort study examines the longterm outcomes of the early biololgical era.
• Chhabra A, Robinson C, Houghton K, et al. Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience. Rheumatology. 2020;59(12):3727–30. https://doi.org/10.1093/rheumatology/keaa118. This study completes a chart review, examining the long-term outcomes of the biologic era.
Giannini EH, Ilowite NT, Lovell DJ, et al. Long-term safety and effectiveness of etanercept in children with selected categories of juvenile idiopathic arthritis. Arthritis Rheum. 2009;60(9):2794–804. https://doi.org/10.1002/art.24777.
Agency for Clinical Innovation. Model of care for the NSW Paediatric Rheumatology network. Musculoskeletal NetworkMay 2013. Chatswood: NSW. https://aci.health.nsw.gov.au/__data/assets/pdf_file/0011/183656/HS13-027_ACI_PaedRheum_web.pdf Accessed 1 March 1 2023.
Davies K, Cleary G, Foster H, et al. BSPAR Standards of Care for children and young people with juvenile idiopathic arthritis. Rheumatology. 2010;49(7):1406–8. https://doi.org/10.1093/rheumatology/kep460.
Onel KB, Horton DB, Lovell DJ, et al. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis. Arthritis Care Res. 2022;74(4):521–37. https://doi.org/10.1002/acr.24853.
Onel KB, Horton DB, Lovell DJ, et al. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: recommendations for nonpharmacologic therapies, medication monitoring, immunizations, and imaging. Arthritis Care Res (Hoboken). 2022;74(4):505–20. https://doi.org/10.1002/acr.24839.
Cellucci T, Guzman J, Petty RE, et al. Management of Juvenile Idiopathic Arthritis 2015: a position statement from the Pediatric Committee of the Canadian Rheumatology Association. J Rheumatol. 2016;43(10):1773. https://doi.org/10.3899/jrheum.160074.
Arthritis Australia. Managing JIA. 2017. https://arthritisaustralia.com.au/managing-arthritis/arthritis-and-children/managing_jia/ Accessed 4 May 2023.
Giancane G, Consolaro A, Lanni S, Davì S, Schiappapietra B, Ravelli A. Juvenile Idiopathic Arthritis: diagnosis and treatment. Rheumatol Ther. 2016;3(2):187–207.
Fingerhutova S, Saifridova M, Vranova M, et al. Is there an evidence for the role of multidisciplinary team in the management of active juvenile idiopathic arthritis? Pediatr Rheumatol Online J. 2014;12(Suppl 1):P177–P177.
Fellas A, Singh-Grewal D, Chaitow J, Santos D, Clapham M, Coda A. Effect of preformed foot orthoses in reducing pain in children with juvenile idiopathic arthritis: a multicentre randomized clinical trial. Rheumatology. 2022;61(6):2572–82. https://doi.org/10.1093/rheumatology/keab765.
Hendry G, Watt G, Brandon M, et al. The effectivenes of a multidisciplinary foot care program for children and adolescents with Juvenile Idiopathic Arthritis: an exploratory trial. J Rehabil Med. 2013;45(5):467–76. https://doi.org/10.2340/16501977-1130.
• Rypdal V, Glerup M, Songstad NT, et al. Uveitis in Juvenile Idiopathic Arthritis: 18-year outcome in the population-based Nordic Cohort study. Ophthalmology. 2021;128(4):598–608. https://doi.org/10.1016/j.ophtha.2020.08.024. This study discusses the screening period for uveitis.
Correll CK, Ditmyer MM, Mehta J, et al. 2015 American College of Rheumatology workforce study and demand projections of pediatric rheumatology workforce, 2015–2030. Arthritis Care Res (Hoboken). 2022;74(3):340–8. https://doi.org/10.1002/acr.24497.
Henrickson M. Policy challenges for the pediatric rheumatology workforce: Part III. the international situation. Pediatr Rheumatol Online J. 2011;9:26–26. https://doi.org/10.1186/1546-0096-9-26.
Tangcheewinsirikul S, Tang S-P, Smith N, et al. Delivery of paediatric rheumatology care: a survey of current clinical practice in Southeast Asia and Asia-Pacific regions. Pediatr Rheumatol. 2021;19(1):11. https://doi.org/10.1186/s12969-021-00498-1.
Australian Rheumatology Assosiation. ARA Rheumatolgy Workforce Report Februaury 2023. 1–29. https://rheumatology.org.au/Portals/2/Documents/Public/About%20the%20ARA/News%20and%20media/ARA%20Workforce%20Doc_DIGITAL_compressed.pdf?ver=2023-02-16-164318-850 Accessed 10 May 2023.
Scott C, Sawhney S, Lewandowski LB. Pediatric rheumatic disease in lower to middle-income countries: impact of global disparities, ancestral diversity, and the path forward. Rheum Dis Clin North Am. 2022;48(1):199–215. https://doi.org/10.1016/j.rdc.2021.09.001.
Migowa AN, Hadef D, Hamdi W, et al. Pediatric rheumatology in Africa: thriving amidst challenges. Pediatr Rheumatol. 2021;19(1):69. https://doi.org/10.1186/s12969-021-00557-7.
Jianghong DJZ, Xiaohua T, Shipeng L, et al. Development history, current situation and prospect of pediatric rheumatology in China. Asian Pac J Child Health Pediactrics 2023;6:4–13. Retrieved from https://www.apjpch.com/?page=article&number=270&article=Development%20history,%20current%20situation%20and%20prospect%20of%20pediatric%20rheumatology%20in%20China Accessed 3 February 2024.
Barber CEH, Lix LM, Lacaille D, et al. Testing population-based performance measures identifies gaps in juvenile idiopathic arthritis (JIA) care. BMC Health Serv Res. 2019;19(1):572. https://doi.org/10.1186/s12913-019-4379-4.
Ooi PL, Shek LP-C. Paediatric rheumatology: a subspecialty in its infancy that is making leaps and bounds. Singap Med J. 2014;55(5):242–3. https://doi.org/10.11622/smedj.2014074.
Bin Dahman HA. Challenges in the diagnosis and management of Pediatric Rheumatology in the developing world: lessons from a newly established clinic in Yemen. Sudan J Paediatr. 2017;17(2):21–9. https://doi.org/10.24911/SJP.2017.2.2.
Spencer CH. Why should pediatric rheumatology be recognized as a separate subspecialty: an open letter to medical councils and government agencies. Pediatr Rheumatol Online J. 2007;5:21–21. https://doi.org/10.1186/1546-0096-5-21.
Henrickson M. Policy challenges for the pediatric rheumatology workforce: Part I Education and economics. Pediatr Rheumatol. 2011;9(1):23. https://doi.org/10.1186/1546-0096-9-24.
Schultz K, Hennard T, Gregg B, Klein M, Real F, Huggins J. Addressing the pediatric rheumatology workforce shortage: is early exposure enough? [abstract]. Arthritis Rheumatol. 2020;72. https://acrabstracts.org/abstract/addressing-the-pediatric-rheumatology-workforce-shortage-is-early-exposure-enough/ Accessed 4 May 2023.
Srinivasalu H, Riebschleger M. Medical education in pediatric rheumatology-unique challenges and opportunities. Clin Rheumatol. 2020;39(3):643–50. https://doi.org/10.1007/s10067-019-04746-4.
Schujovitzky D, Ziv A, Heshin Bekenstein M, et al. Measuring the knowledge of juvenile idiopathic arthritis among Israeli paediatricians and paediatric orthopaedic surgeons: what needs to be improved? Clin Exp Rheumatol. 2022;40(11):2188–93. https://doi.org/10.55563/clinexprheumatol/7j9es3.
Aoust L, Rossi-Semerano L, Koné-Paut I, Dusser P. Time to diagnosis in juvenile idiopathic arthritis: a French perspective. Orphanet J Rare Dis. 2017;12(1):43. https://doi.org/10.1186/s13023-017-0586-4.
Lapin W, Kutac C, Guttman-Lapin D, Brown A, Muscal E, Seeborg F. School nurse education for juvenile idiopathic arthritis [abstract]. Arthritis Rheumato. 2019;71. https://acrabstracts.org/abstract/school-nurse-education-for-juvenile-idiopathic-arthritis/ Accessed 4 May 2023.
Shoop-Worrall SJW, Moull L, McDonagh JE, Hyrich KL. The role of age in delays to rheumatological care in juvenile idiopathic arthritis. J Rheumatol. 2022;49(9):1037. https://doi.org/10.3899/jrheum.211316.
•• Consolaro A, Giancane G, Alongi A, et al. Phenotypic variability and disparities in treatment and outcomes of childhood arthritis throughout the world: an observational cohort study. Lancet Child Adolesc Health. 2019;3(4):255–63. https://doi.org/10.1016/S2352-4642(19)30027-6. This paper identifies a link between delayed diagnosis and disease damage.
GBD 2015 Child Mortality Collaborators. Global, regional, national, and selected subnational levels of stillbirths, neonatal, infant, and under-5 mortality, 1980-2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet. 2016;388(10053):1725–74. https://doi.org/10.1016/S0140-6736(16)31575-6.
Kassebaum N, Kyu HH, Zoeckler L, Olsen HE, et al. Child and adolescent health from 1990 to 2015: findings from the global burden of diseases, injuries, and risk factors 2015 study. JAMA Pediatr. 2017;6:573–92. https://doi.org/10.1001/jamapediatrics.2017.0250.
Scott C, Chan M, Slamang W, et al. Juvenile arthritis management in less resourced countries (JAMLess): consensus recommendations from the Cradle of Humankind. Clin Rheumatol. 2019;38(2):563–75. https://doi.org/10.1007/s10067-018-4304-y.
Department of Health. National Action Plan forthe Health of Children and Young People 2020 – 2030. 2019:1–40. Commonwealth of Australia. https://www.health.gov.au/sites/default/files/documents/2021/04/national-action-plan-for-the-health-of-children-and-young-people-2020-2030-national-action-plan-for-the-health-of-children-and-young-people-2020-2030.pdf Accessed 4 February 2024.
Coda A, Sculley D, Santos D, et al. Harnessing interactive technologies to improve health outcomes in juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2017;15(1):40. https://doi.org/10.1186/s12969-017-0168-y.
Royal Australian College of Physicians. Physicians and integrated care discussion paper, February 2018. https://www.racp.edu.au/docs/default-source/advocacy-library/integrated-care-physicians-supporting-better-patient-outcomes-discussion-paper.pdf Accessed 4 February 2024.
Perdue A, Mullett C, Umer A, et al. Utility of telemedicine in pediatric rheumatology during the COVID-19 pandemic. Pediatric Rheumatology. 2021;19(1):148. https://doi.org/10.1186/s12969-021-00624-z. Accessed 4 February 2024.
Hayward K, Goy I, Toth M, et al. Assessment survey of critical data element completion in telemedicine visits for juvenile idiopathic arthritis in the post-pandemic period [abstract]. Arthritis Rheumatol. 2023:75(supp 19). https://acrabstracts.org/abstract/needs-assessment-survey-of-critical-data-element-completion-in-telemedicine-visits-for-juvenile-idiopathic-arthritis-in-the-post-pandemic-period/ Accessed 5 February 2024.
Goh YI, Bullock DR, Taylor J, et al. Exploring pediatric tele-rheumatology practices during COVID-19: a survey of the PRCOIN network. Front Pediatr. 2021;9:642460. https://doi.org/10.3389/fped.2021.642460.
• Shenoi S, Hayward K, Curran ML, et al. Telemedicine in pediatric rheumatology: this is the time for the community to embrace a new way of clinical practice. Pediatr Rheumatol. 2020;18:85. https://doi.org/10.1186/s12969-020-00476-z. This study provides a guide for delivering telemedicine.
Turnbull A, Sculley D, Escalona-Marfil C, et al. Comparison of a mobile health electronic visual analog scale app with a traditional paper visual analog scale for pain evaluation: Cross-Sectional Observational Study. J Med Internet Res. 2020;22(9):e18284. https://doi.org/10.2196/18284.
Lupton D. Health promotion in the digital era: a critical commentary. Health Promot Int. 2014;30(1):174–83. https://doi.org/10.1093/heapro/dau091.
Mosa ASM, Yoo I, Sheets L. A systematic review of healthcare applications for smartphones. BMC Med Inform Decis Mak. 2012;12:67–67. https://doi.org/10.1186/1472-6947-12-67.
Termeh VR, Niaraki AS. Design and implementation of ubiquitous health system (U-Health) using Smart-watches sensors. ISPRS - Int Arch Photogramm Remote Sens Spatial Inf Sci 2015:07-612. https://doi.org/10.5194/isprsarchives-XL-1-W5-607-2015.
Butler S, Sculley D, Santos D, et al. Effectiveness of eHealth and mHealth interventions supporting children and young people living with juvenile idiopathic arthritis: systematic review and meta-analysis. J Med Internet Res. 2022;24(2):e30457. https://doi.org/10.2196/30457.
Stinson JN, Jibb LA, Lalloo C, et al. Comparison of average weekly pain using recalled paper and momentary assessment electronic diary reports in children with arthritis. Clin J Pain. 2014;30(12):1044–50. https://doi.org/10.1097/AJP.0000000000000072.
• Doeleman MJH, de Roock S, Buijsse N, et al. Monitoring patients with juvenile idiopathic arthritis using health-related quality of life. Pediatr Rheumatol. 2021;19(1):40. https://doi.org/10.1186/s12969-021-00527-z. This study focuses on the EQ-5D-Y-5 L to identify treatment adjustments.
Haverman L, van Rossum MA, van Veenendaal M, et al. Effectiveness of a web-based application to monitor health-related quality of life. Pediatrics. 2013;131(2):e533-543. https://doi.org/10.1542/peds.2012-0958.
Butler S, Sculley D, Santos D, Girones X, Singh-Grewal D, Coda A. Using digital health technologies to monitor pain, medication adherence and physical activity in young people with juvenile idiopathic arthritis: a feasibility study. Healthcare. 2024;12(3):392. https://doi.org/10.3390/healthcare12030392.
Grande SW, Longacre MR, Palmblad K, et al. Empowering young people living with juvenile idiopathic arthritis to better communicate with families and care teams: content analysis of semistructured interviews. JMIR Mhealth Uhealth. 2019;7(2):e10401. https://doi.org/10.2196/10401.
Funding
Open Access funding enabled and organized by CAUL and its Member Institutions
Author information
Authors and Affiliations
Contributions
Sonia Butler: Conceptualization, data curation, writing; Sonia Butler, Dean Sculley, Derek Santos, Xavier Girones, Davinder Singh-Grewal, Andrea Coda: Formal analysis, validation, reviewing and editing.
Corresponding author
Ethics declarations
Ethics Approval
This review did not require an ethical board approval because it was conducted as a literature review.
Consent to Participate
Gaining consent was not needed because this study was conducted as a literature review.
Conflict of Interest
The authors declare no competing interests.
Human and Animal Rights and Informed Consent
This review article refers to one studies with human subjects performed previously by the authors. Written informed consent was gained from all subjects involved in this study. Written informed consent was also obtained from all subjects, to publish the study results (Hunter New England Ethics Committee approval number: 2019/ETHO1035).
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.
About this article
Cite this article
Butler, S., Sculley, D., Santos, D. et al. Paediatric Rheumatology Fails to Meet Current Benchmarks, a Call for Health Equity for Children Living with Juvenile Idiopathic Arthritis, Using Digital Health Technologies. Curr Rheumatol Rep 26, 214–221 (2024). https://doi.org/10.1007/s11926-024-01145-w
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11926-024-01145-w