Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood, affecting more than 300,000 children. JIA differs from adult forms of arthritis in a number of important ways that add to the challenges of management.1,2
Children with JIA have fewer comorbidities than adults with rheumatic diseases, and they have better responses to therapies; however, their musculoskeletal structure is continually growing and changing, requiring special care in administering injectable therapies. JIA develops in girls at twice the rate of boys, particularly the oligoarticular type, involving inflammation of multiple joints, and the special emotional and developmental needs of these patients should be taken into account when devising treatment strategies.2,3 Because JIA is a chronic condition often requiring long-term treatment, adherence is a major issue among children and adolescents, who are both more forgetful and more resistant to receiving therapy.
A 2013 article by Philip J. Kahn, MD, of the New York University School of Medicine, Division of Pediatric Rheumatology, laid out 8 distinct subgroups of JIA onset (including most commonly, extended-oligoarticular JIA, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis) that occur before age 16 with a duration of at least 6 weeks and no apparent cause.2
For most adolescents with JIA, the disease is likely to progress into adulthood and, if not treated effectively early on, can produce significant physical disability and impairment to educational, psychosocial, and physical development in later life.4,5 Technological advances can be helpful in JIA and are particularly well suited to younger people in general, who are more comfortable with device technologies and more likely to adopt them.
Studies have indicated that musculoskeletal ultrasound (MSUS) can be useful in monitoring disease progression of JIA and in needle guidance for injectable therapies; however, the full role of MSUS in JIA has not been established.
Synovial inflammation is generally present for long periods of time in JIA, which leads to cartilage and bone damage that impair daily function and quality of life.3 Several literature reviews have indicated that MSUS imaging helps distinguish joint synovitis from tenosynovitis, and may help detect enthesitis as well as bone and cartilage and erosions in children.3,6,7
Magni-Manzoni and colleagues7 found that young patients determined to be in remission still had a high frequency of subclinical synovitis detected on magnetic resonance imaging (MRI) and ultrasound, but noted that “more information from healthy children is needed to enable differentiation of the bone and cartilage abnormalities that reflect damage from those that are part of normal development using MRI or ultrasonography.”
As an image-guidance tool for intra-articular corticosteroid injections, MSUS can be invaluable to rheumatologists, both in the detection of inflammation requiring treatment and in needle placement to optimize therapeutic value.4 Most pediatric rheumatologists are not experienced in performing ultrasound, however, limiting its use in current practice.4,8 The changing anatomies of children and adolescents also pose challenges to differentiating JIA pathophysiology from natural growth patterns, leading Lanni and colleagues to conclude that, “a thorough knowledge of ultrasound anatomy of joints in growing children is necessary to interpret ultrasound findings in JIA patients.”9
Devices and Apps
Increasingly, researchers have looked to interactive devices to improve management of JIA. A group of investigators from the United Kingdom reported recently on a smartphone app (called the JIApp), designed to “minimize the impact of JIA on the physical and psychosocial well-being and development of young people.”8
In phase 1 of development, the team analyzed a number of health management behaviors that could be readily tracked by smartphone and, with input from patients with JIA and healthcare providers, they determined which features would best support adhering with therapy and coping with JIA symptoms while collecting the most informative data. In phase 2, they consulted with the same groups for protocols to implement the app into clinical practice.
“We are hoping the use of the app will lead to less frequent consultations and appointments by allowing young people to self-manage their JIA more effectively and to also engage remotely with healthcare,” chief investigator Despina Eleftheriou, Great Ormond Street Institute of Child Health, Infection, Immunity, and Physiological Medicine in London, UK, explained to Rheumatology Advisor.
The 3 primary themes of the JIApp were allowing for self-monitoring of JIA symptoms, general well-being, activities, and sleep; enhancing treatment adherence through tracking, reminders, and incentives; and providing education and support. “The initial aim is to enable better self-management for patients, but in the long term, the vision is to have an alert system for notifying healthcare professionals,” Dr. Eleftheriou said.
Both young adults with JIA and healthcare providers were reported to consider the app highly acceptable and useful in a pilot study, which the authors intend to follow up with feasibility studies.8 “We are already working with the National Health Service in pediatric settings to get this adopted,” Dr. Eleftheriou added.
The Potential of New Technologies in JIA
A 2017 review by Coda et al.10 concluded that emerging interactive technologies have great potential to improve management strategies in JIA, including monitoring of disease progression (even during clinically defined remission), improving adherence, encouraging physical activity, and engaging children and adolescents in their own JIA management process.
“These smart devices can gather a very large number of data,” Dr. Coda told Rheumatology Advisor, adding that “it would certainly be valuable to be able to record pain level, physical activity, and adherence to the prescribed medication. The interaction would typically depend, based on the severity of the [patient’s] condition and age.”
The potential of these technologies to improve the quality of care in JIA is immense, according to Dr. Coda. “Interactive online/cloud technology might be of help to ensure that different health outcome measures in JIA [can] be collected anytime and anywhere by the different members of the multidisciplinary pediatric rheumatology team,” she said. She and her colleagues will soon begin a new randomized controlled trial to identify which products might work best.
At the same time, however, Dr. Coda warned that new technologies that share data within registries and in real time present unique data protection and privacy issues that will first need to be resolved before these interactive devices and apps can be introduced into general clinical practice.
- Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369:767-778.
- Kahn PJ. Juvenile idiopathic arthritis: what the clinician needs to know. Bull Hosp Jt Dis. 2013;7:194-199.
- Magni-Manzoni S. Ultrasound in juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2016;14:33.
- Vidqvist K-L, Malin M, Varjolahti-Lehtinen T, Korpela MM. Disease activity of idiopathic juvenile arthritis continues through adolescence despite the use of biologic therapies. Rheumatology (Oxford). 2013;52:1999-2003.
- Selvaag AM, Aulie HA, Lilleby V, Flatø B. Disease progression into adulthood and predictors of long-term active disease in juvenile idiopathic arthritis. Ann Rheum Dis. 2016;75:190-195.
- Collado P, Jousse-Joulin S Alcalde M, Naredo E, D’Agostino MA. Is ultrasound a validated imaging tool for the diagnosis and management of synovitis in juvenile idiopathic arthritis? A systematic literature review. Arthritis Care Res. 2012;64:1011-1119.
- Magni-Manzoni S, Malattia C, Lanni S, Ravelli A. Advances and challenges in imaging in juvenile idiopathic arthritis. Nat Rev Rheumatol. 2012;8:329-336.
- Cai RA, Beste D, Chaplin H, et al. Developing and evaluating JIApp: acceptability and usability of a smartphone app system to improve self-management in young people with juvenile idiopathic arthritis. JMIR Mhealth Uhealth. 2017;5:e121.
- Lanni S, Wood M, Ravelli A, Magni Manzoni S, Emery P, Wakefield RJ. Towards a role of ultrasound in children with juvenile idiopathic arthritis. Rheumatology (Oxford). 2013;52:413-420.
- Coda A, Sculley D, Santos D, et al. Harnessing interactive technologies to improve health outcomes in juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2017;15:40.
This article originally appeared on Rheumatology Advisor