Search

Edward J Oberle, Julia G Harris, James W VerbskyDepartment of Pediatrics, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI, USAAbstract: Juvenile idiopathic arthritis (JIA) is a group of disorders characterized by arthritis persisting for at least 6 weeks with onset before the age of 16 years. Within this cluster of conditions, the polyarticular form (involving more than four joints within the first 6 months) is further divided based on the presence of rheumatoid factor. Children with polyarticular JIA pose unique diagnostic and therapeutic challenges compared to children with involvement of fewer joints. Polyarticular JIA patients tend to have a more refractory course and therefore are at increased risk for joint damage, resulting in poorer functional outcomes and decreased quality of life. Although the ability to treat this disorder continues to improve, especially with the advent of biologic agents, there is still much about the epidemiology and pathogenesis of polyarticular JIA that is unknown. The epidemiology of polyarticular JIA varies worldwide with a vast difference in reported cases between different global regions as well as within individual countries. Several genetic risk loci have been identified conferring increased susceptibility to JIA, many within the human leukocyte antigen region. Beyond the genome, environmental factors also seem to contribute to the etiology of polyarticular JIA. This review article will focus on the epidemiology and current treatments of polyarticular JIA and briefly discuss genetic and environmental influences on the pathogenesis of JIA as well as new and emerging therapies.Keywords: juvenile arthritis, polyarticular, epidemiology, treatment, rheumatology

Introduction: The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network's global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to 80% by the end of 2023., Methods: Twenty-three hospitals participate in PR-COIN, with over 7,200 active patients with JIA. The disease activity outcome measures include active joint count, physician global assessment of disease activity, and measures related to validated composite disease activity scoring systems including inactive or low disease activity by the 10-joint clinical Juvenile Arthritis Disease Activity Score (cJADAS10), inactive or low disease activity by cJADAS10 at 6 months post-diagnosis, mean cJADAS10 score, and the American College of Rheumatology (ACR) provisional criteria for clinical inactive disease. Data is collated to measure network performance, which is displayed on run and control charts. Network-wide interventions have included pre-visit planning, shared decision making, self-management support, population health management, and utilizing a Treat to Target approach to care., Results: Five outcome measures related to disease activity have demonstrated significant improvement over time. The percent of patients with inactive or low disease activity by cJADAS10 surpassed our goal with current network performance at 81%. Clinical inactive disease by ACR provisional criteria improved from 46% to 60%. The mean cJADAS10 score decreased from 4.3 to 2.6, and the mean active joint count declined from 1.5 to 0.7. Mean physician global assessment of disease activity significantly improved from 1 to 0.6., Conclusions: PR-COIN has shown significant improvement in disease activity metrics for patients with JIA. The network will continue to work on both site-specific and collaborative efforts to improve outcomes for children with JIA with attention to health equity, severity adjustment, and data quality., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Harris, Bingham, Vora, Yildirim-Toruner, Batthish, Bullock, Burnham, Fair, Ferraro, Ganguli, Gilbert, Gottlieb, Halyabar, Hazen, Laxer, Lee, Liu, Lovell, Mannion, Oberle, Pan, Shishov, Weiss and Morgan.)

Objective: To describe the selection, development, and implementation of quality measures (QMs) for juvenile idiopathic arthritis (JIA) by the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN), a multihospital learning health network using quality improvement methods and leveraging QMs to drive improved outcomes across a JIA population since 2011., Methods: An American College of Rheumatology-endorsed multistakeholder process previously selected initial process QMs. Clinicians in PR-COIN and parents of children with JIA collaboratively selected outcome QMs. A committee of rheumatologists and data analysts developed operational definitions. QMs were programmed and validated using patient data. Measures are populated by registry data, and performance is displayed on automated statistical process control charts. PR-COIN centers use rapid-cycle quality improvement approaches to improve performance metrics. The QMs are revised for usefulness, to reflect best practices, and to support network initiatives., Results: The initial QM set included 13 process measures concerning standardized measurement of disease activity, collection of patient-reported outcome assessments, and clinical performance measures. Initial outcome measures were clinical inactive disease, low pain score, and optimal physical functioning. The revised QM set has 20 measures and includes additional measures of disease activity, data quality, and a balancing measure., Conclusion: PR-COIN has developed and tested JIA QMs to assess clinical performance and patient outcomes. The implementation of robust QMs is important to improve quality of care. PR-COIN's set of JIA QMs is the first comprehensive set of QMs used at the point-of-care for a large cohort of JIA patients in a variety of pediatric rheumatology practice settings., (© 2023 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Background: Teach Back (TB) is recommended to assess and ensure patient understanding, thereby promoting safety, quality, and equity. There are many TB trainings, typically lacking assessment tools with validity evidence. We used a pediatric resident competency-based communication curriculum to develop initial validity evidence and refinement recommendations for a Teach-back Observation Tool (T-BOT)., Objective: This study aimed to develop initial validity evidence for a refined T-BOT and provide guidance for further enhancements to improve essential TB skills training among pediatric residents., Methods: After an interactive health literacy (HL) training, residents participated in recorded standardized patient (SP) encounters. Raters developed T-BOT scoring criteria, then scored a gold standard TB video and resident SP encounters. For agreement, Fleiss' Kappa was computed for >2 raters, and Cohen's Kappa for two raters. Percent agreement and intraclass correlation (ICC) were calculated. Statistics were calculated for gold standard (GS) and TB items overall for all six raters, and for five faculty raters. Agreement was based on Kappa: no agreement (≤0), none to slight (0.01-0.20), fair (0.21-0.40), moderate (0.41-0.60), substantial (0.61-0.80), almost perfect (0.81-1.00)., Key Results: For six raters, Kappa for the GS was 0.554 (moderate agreement) with 71.4% agreement; I CC = .597; for SP encounters, it was 0.637 (substantial) with 65.4% agreement; I CC = .647. Individual item agreement for SP encounters average was 0.605 (moderate), ranging from 0.142 (slight) to 1 (perfect). For five faculty raters, Kappa for the GS was 0.779 (substantial) with 85.7% agreement; I CC = .824; for resident SP encounters, it was 0.751 (substantial), with 76.9% agreement; I CC = .759. Individual item agreement on SP encounters average was 0.718 (substantial), ranging from 0.156 (slight) to 1 (perfect)., Conclusion: We provide initial validity evidence for a modified T-BOT and recommendations for improvement. With further refinements to increase validity evidence, accompanied by shared understanding of TB and rating criteria, the T-BOT may be useful in strengthening approaches to teaching and improving essential TB skills among health care team members, thereby increasing organizational HL and improving outcomes. [ HLRP: Health Literacy Research and Practice . 2023;7(4):e187-e196. ].

Objective: To determine the frequency of subclinical synovitis on musculoskeletal ultrasonography (MSUS) in juvenile idiopathic arthritis (JIA) and correlate patient- and provider-reported outcome measures with MSUS synovitis., Method: JIA patients with an active joint count (AJC) of >4 underwent a 42-joint MSUS performed at baseline and 3 months. B-mode and power Doppler images were obtained and scored (range 0-3) for each of the 42 joints. Outcomes evaluated included physician global assessment of disease activity (PhGA), patient global assessment of disease activity (PtGA), patient pain, Childhood Health Assessment Questionnaire (C-HAQ), and AJC. Subclinical synovitis was defined as synovitis detected by MSUS only. Generalized estimation equations were used to test the relationship between clinical arthritis (positive/negative) and subclinical synovitis (positive/negative). Spearman's correlation coefficients (r s ) were calculated to determine the association between MSUS synovitis and patient- and physician-reported outcomes., Results: In 30 patients, subclinical synovitis was detected in 30% of joints. Clinical arthritis of the fingers, wrists, and knee joints was significantly associated with MSUS synovitis in these joints. PtGA and the C-HAQ had a moderate (r s  = 0.44, P = 0.014) to weak (r s  = 0.37, P = 0.045) correlation with MSUS synovitis. There was a statistically significant strong correlation between MSUS synovitis and PhGA (r s  = 0.61, P = 0.001), but a weak correlation with AJC (r s  = 0.37, P = 0.048) at the follow-up visit., Conclusion: Subclinical synovitis was commonly observed in this cohort of JIA patients. The fair-to-moderate correlation of MSUS synovitis with patient- and provider-reported outcomes suggests that MSUS assesses a different, possibly more objective, domain not determined by traditional JIA outcome measurements., (© 2022 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Objective: To develop and initially validate a comprehensive pediatric musculoskeletal ultrasound (MSUS) joint-specific scoring system, and to determine the minimum number of joints needed to identify active disease., Methods: A semiquantitative scoring system was developed by consensus and initially validated by interrater reliability using intraclass correlation coefficients (ICCs). Subsequently, newly diagnosed juvenile idiopathic arthritis patients with an active joint count of >4 had a 42-joint MSUS performed at baseline and 3 months using this protocol. A minimum set of joints needed to identify all patients with synovitis on MSUS was obtained through a data reduction process. Spearman's correlation (r s ) was calculated to determine the association between MSUS findings and clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10). Standardized response means (SMRs) were used to assess change over time., Results: The final joint-specific scoring system revealed an excellent interrater reliability (ICC 0.81-0.96) for all joints. Thirty patients were enrolled. Scanning 5 joints bilaterally (wrists, second and third metacarpophalangeal joints, knees and ankles) captured 100% of children with B-mode synovitis and had moderate correlation with the cJADAS10 at baseline (r s  = 0.45). Mean ultrasound scores at baseline and follow-up were 28.3 and 22.3, with an SRM of 0.69 (P = 0.002) for 42 joints, and 36 and 27.7, with an SRM of 0.76 (P = 0.003) for the reduced joints, respectively., Conclusion: A limited MSUS examination called musculoskeletal ultrasound in childhood arthritis limited examination (MUSICAL) captures all patients with active synovitis, and our new joint-specific scoring system is highly reliable and sensitive to change., (© 2021 American College of Rheumatology.)

Background: Adolescents with chronic disease engage in sexual activity similar to their healthy peers, with generally low utilization of contraception. Adolescents with rheumatic diseases prescribed teratogenic medications may be at risk for unplanned pregnancy., Methods: Using structured quality improvement (QI) methods with behavior economic (BE) principles, a multidisciplinary team aimed to implement pregnancy prevention processes for females on high-risk medications. We leveraged BE-inspired interventions including improved accessibility of consents, utilizing distinctly colored consent forms, real-time reminders, peer comparison, and audit and feedback. Our primary aim was to increase the number of days between pregnancies for postmenarcheal females followed in rheumatology clinics who were taking teratogenic medications. Phase 1 focused on annual consenting of female adolescents prescribed teratogenic drugs. Phase 2 emphasized sexual history screening and pregnancy prevention planning at every clinic visit for females ≥12 years on teratogenic medications., Results: We increased the days between pregnancies for female adolescents prescribed teratogenic medications from 52 days to >900 days by using QI methodology with BE strategies. In phase 1, annual consents for postmenarcheal patients on teratogenic medications improved from 0% in 2017 to 95% in 2021. In phase 2, sexual history screening and pregnancy prevention planning at every clinic visit improved from 2% in 2019 to over 78% in 2021., Conclusions: A multiphase, multidisciplinary QI project with integration of behavior economic strategies can improve patient and caregiver counseling to prevent unplanned pregnancies for adolescents on teratogenic medications., (Copyright © 2023 by the American Academy of Pediatrics.)

This review highlights the increasing evidence from the last few years supporting the use of musculoskeletal ultrasonography (MSUS) in the evaluation and management of patients with pediatric rheumatic diseases, particularly focusing on juvenile idiopathic arthritis. Recently developed definitions for the sonographic appearance of healthy and pathologic joints in children are discussed. Further topics explored include how MSUS enhances the diagnosis of inflammatory joint disease (synovitis, enthesitis, tenosynovitis), including the detection of subclinical synovitis. There is a brief summary on the use of ultrasonography in the evaluations of myositis, Sjögren syndrome, and scleroderma., Competing Interests: Disclosure E.J. Oberle and P. Vega-Fernandez are mentors for USSONAR. The other authors have nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background/objective: A North American rheumatology consensus on tiered-mastery designation for anatomic views was developed in 2011 for course and fellowship teaching. This study updates the lower extremity joint scanning protocols aiming to inform musculoskeletal ultrasound curriculum development for the American College of Rheumatology affiliated Fellowship Programs., Methods: Three Delphi rounds were conducted to reach consensus for tiered-level mastery designation for hip, knee, ankle, and foot scanning views. The survey was disseminated (Qualtrics™) to 101 potential participants with ultrasound teaching experience. High agreement was defined as ≥ 85% consensus and final tier designation as having >50% agreement for the preferred tier. Response changes were evaluated by McNemar's chi-square test., Results: Consensus regarding tier designations was reached for 80% of the views. Three knee views (anterior transverse suprapatellar, medial, and lateral longitudinal) and 2 ankle views (anterior and posterior transverse) achieved upgrades to tier 1 from 2. The transverse sacroiliac hip joint was downgraded from tier 2 to 3. The lateral longitudinal hip view was added with a tier 1 designation., Conclusion: Updated scanning protocols support modifications reflecting current scanning methods delivered by North American rheumatologists performing point of care ultrasound that may inform educators involved in rheumatology ultrasound. Key Points • The anterior transverse suprapatellar, medial, and lateral longitudinal knee views; the anterior and posterior transverse ankle views; and the lateral longitudinal view hip view were perceived as important to master and perform routinely. • The transverse sacroiliac joint view was suggested to be performed based on practice focus., (© 2021. International League of Associations for Rheumatology (ILAR).)

Objective: Magnetic resonance imaging (MRI) is pivotal in the assessment of early sacroiliitis in children. We aimed to evaluate the agreement between local radiology reports and central imaging reviewers for active inflammation and structural damage at the sacroiliac (SI) joints., Methods: Eight hospitals each contributed up to 20 cases of consecutively imaged children and adolescents with juvenile idiopathic arthritis and suspected sacroiliitis. Studies were independently reviewed by 3 experienced musculoskeletal pediatric radiologists. Local assessments of global impression and lesions were coded from the local radiology reports by 2 study team members. Test properties of local reports were calculated using the central imaging team's majority as the reference standard., Results: For 120 evaluable subjects, the median age was 14 years, half of the cases were male, and median disease duration at the time of imaging was 0.8 years (interquartile range 0-2). Sensitivity of local reports for inflammation was high, 93.5% (95% confidence interval [95% CI] 78.6-99.2), and specificity was moderate, 69.7% (95% CI 59.0-79.0), but positive predictive value (PPV) was low, 51.8% (95% CI 38.0-65.3). Twenty-seven cases (23%) had active inflammation reported locally but rated normal at the central reading, 19 (70%) with subsequent medication changes. The sensitivity of local reports detecting structural damage was low, 45.7% (95% CI 28.8-63.4), and specificity was high, 88.2% (95% CI 79.4-94.2); PPV was low, 61.5% (95% CI 40.6-79.8)., Conclusion: Substantial variation exists in the interpretation of inflammatory and structural lesions at the SI joints in children. To reliably identify pathology, additional training in the MRI appearance of the maturing SI joint is greatly needed., (© 2020, American College of Rheumatology.)

Background: While musculoskeletal ultrasound (MSUS) use in pediatric rheumatology is becoming more common, the majority of pediatric MSUS literature continues to focus on ultrasound findings in healthy children and juvenile idiopathic arthritis with little discussion of other musculoskeletal problems that may mimic arthritis such as joint hypermobility. Chronic joint pain related to hypermobility is a common referral to pediatric rheumatology clinics. Our aim is to describe the musculoskeletal ultrasound (MSUS) characteristics of the knee in a population with joint hypermobility and pain in comparison to control participants., Methods: Participants were recruited into three groups for a case-control study. Case group participants had knee hypermobility and pain symptoms (H + P). Participants in one control group had knee hypermobility without pain symptoms (H-P), and participants in the other control group had no knee hypermobility or pain symptoms (NP). B-mode and Doppler MSUS images were obtained and scored for each knee. Descriptive statistics are used for demographic variables and MSUS findings. Regression analysis is used to evaluate risk of synovial effusion and higher synovial effusion/hypertrophy quantitative score., Results: MSUS assessment was performed on 91 knees of 50 participants. H + P knees were more likely to have positive findings noted on MSUS (94% vs. 70% of H-P and 74% of NP knees, p = 0.043). Patellar tendon hyperemia was more common in H + P knees (52%, vs. 19% among H-P and 23% among NP, p = 0.025). Participants who reported taking scheduled non-steroidal anti-inflammatory drugs (NSAIDs) had an increased risk of synovial effusion (RR = 1.83, 95% CI = 1.07-2.30, p = 0.026) and a trend towards increased risk of a higher synovial effusion/hypertrophy quantitative score (RR = 1.77, 95% CI = 0.92-3.38, p = 0.086)., Conclusions: While positive MSUS findings were frequent in all participants, patellar tendon hyperemia was more frequent in participants with knee hypermobility and pain symptoms. Additionally, reported use of NSAIDs was associated with an increased risk of synovial effusion and higher synovial effusion/hypertrophy quantitative score. Further study should assess correlation between tendon abnormalities and degree of pain symptoms as well as the effect of NSAIDs on MSUS findings.

Background/objectives: Clinically amyopathic juvenile dermatomyositis (CAJDM) is an uncommon but important subset of patients with juvenile dermatomyositis, characterized by pathognomonic cutaneous findings without clinically evident muscle weakness. With limited data available and lack of standardized management guidelines for CAJDM, we sought to describe common features, including early indicators that may be associated with progression of muscle disease, and review the course and treatment of these patients., Methods: A retrospective chart review of patients with CAJDM was conducted at four North American academic centers between the years 2000 and 2015., Results: Twenty-nine patients were included, of whom 21 (72%) were female. After a median follow-up of 4 years (IQR 1.8-5.8 years), 5 of the 29 (17%) patients with CAJDM evolved into classic juvenile dermatomyositis. Median time to develop weakness was 12 months (IQR 8-19 months) after diagnosis. The skin disease of CAJDM patients who did not develop weakness was often found to be recalcitrant with 58% of them requiring multiple systemic therapies to control their cutaneous disease., Conclusion: These results highlight the need for long-term monitoring for the development of myositis in CAJDM and for prospective studies on treatment of recalcitrant skin disease., (© 2021 Wiley Periodicals LLC.)

Objective: The use of musculoskeletal ultrasound is increasing among pediatric rheumatologists. Reliable scoring systems are needed for the objective assessment of synovitis. The aims of this study were to create a standardized and reproducible image acquisition protocol for B-mode and Doppler ultrasound of the pediatric knee, and to develop a standardized scoring system and determine its reliability for pediatric knee synovitis., Methods: Six pediatric rheumatologists developed a set of standard views for knee assessment in children with juvenile arthritis. Subsequently, a comprehensive literature review, practical exercises, and a consensus process were performed. A scoring system for both B-mode and Doppler was then developed and assessed for reliability. Interreader reliability or agreement among a total of 16 raters was determined using 2-way single-score intraclass correlation coefficient (ICC) analysis., Results: Twenty-one views to assess knee arthritis were initially identified. Following completion of practical exercises and subsequent consensus processes, 3 views in both B-mode and Doppler were selected: suprapatellar longitudinal and medial/lateral parapatellar transverse views. Several rounds of scoring and modifications resulted in a final ICC of suprapatellar view B-mode 0.89 (95% confidence interval [95% CI] 0.86-0.92) and Doppler 0.55 (95% CI 0.41-0.69), medial parapatellar view B-mode 0.76 (95% CI 0.68-0.83) and Doppler 0.75 (95% CI 0.66-0.83), and lateral parapatellar view B-mode 0.82 (95% CI 0.75-0.88) and Doppler 0.76 (95% CI 0.66-0.84)., Conclusion: A novel B-mode and Doppler image acquisition and scoring system for assessing synovitis in the pediatric knee was successfully developed through practical exercises and a consensus process. Study results demonstrate overall good-to-excellent reliability., (© 2018, American College of Rheumatology.)

Background: Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript., Methods: The Children's Arthritis and Rheumatology Research Alliance (CARRA) is a North American consortium of pediatric rheumatology health care providers. CARRA members collaborated to determine consensus on typical treatments for JDM patients with skin findings without significant weakness, to develop CTPs for this subgroup of patients. We used a combination of Delphi surveys and nominal group consensus meetings to develop these CTPs., Results: Consensus was reached on patient characteristics and outcome assessment, and CTPs were developed and finalized for patients with skin predominant JDM. Treatment option A included hydroxychloroquine alone, Treatment option B included hydroxychloroquine and methotrexate, and Treatment option C included hydroxychloroquine, methotrexate and corticosteroids., Conclusions: Three CTPs were developed for use in children with skin predominant JDM, which reflect typical treatment approaches. These are not considered to be specific recommendations or standard of care. Using the CARRA network and prospective data collection, we will be able to apply statistical methods in the future to allow comparisons of JDM patients following these consensus treatment plans.

Background: Pediatric patients can present with skin manifestations of dermatomyositis without overt weakness (clinically amyopathic juvenile dermatomyositis [JDM]), but it is unclear how often this happens and how often they have subclinical muscle inflammation., Objective: Our goal was to determine the frequency of clinically amyopathic JDM and the frequency with which a thorough evaluation uncovers subclinical myositis at a single institution., Methods: A retrospective review was performed of 46 patients diagnosed with JDM at Children's Hospital of Wisconsin., Results: Of 46 patients presenting with skin findings consistent with dermatomyositis, 10 patients (21.7%) did not have evidence of muscle involvement on history or exam, and these tended to be the younger patients. Of these 10, only 2 (4% of all the JDM patients) were truly amyopathic upon further evaluation (all five muscle enzymes [aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, creatine kinase and aldolase], magnetic resonance imaging [MRI], muscle biopsy). In our series, muscle biopsy was not helpful in identifying subclinical myositis. In contrast, MRI did uncover subclinical muscle disease., Conclusion: These data suggest that truly amyopathic JDM is rare and that a thorough workup that includes all five muscle enzymes and MRI may uncover occult myositis., (© 2016 Wiley Periodicals, Inc.)

Calcinosis is one of the hallmark sequelae of juvenile dermatomyositis (JDM), and despite recent progress in the therapy of JDM, dystrophic calcification still occurs in approximately one third of patients. This review discusses our current, albeit limited, understanding of risk factors for the development of calcinosis in JDM, as well as approaches to assessment, and current views on its pathogenesis. Anecdotal approaches to treating calcinosis associated with JDM, including both anti-inflammatory therapies and agents aimed at inhibiting the deposition of calcium hydroxyapatite, are reviewed. An improved understanding of the pathogenesis of calcinosis, the establishment of standardized measurement tools to assess calcinosis, and randomized controlled trials employing more sensitive outcome measures are needed to develop efficacious therapies for this often disabling complication.