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Artificial intelligence algorithms, with roots extending into the past but experiencing a resurgence and evolution in recent years due to their superiority over traditional methods and contributions to human capabilities, have begun to make their presence felt in the field of pediatric rheumatology. In the ever-evolving realm of pediatric rheumatology, there have been incremental advancements supported by artificial intelligence in understanding and stratifying diseases, developing biomarkers, refining visual analyses, and facilitating individualized treatment approaches. However, like in many other domains, these strides have yet to gain clinical applicability and validation, and ethical issues remain unresolved. Furthermore, mastering different and novel terminologies appears challenging for clinicians. This review aims to provide a comprehensive overview of the current literature, categorizing algorithms and their applications, thus offering a fresh perspective on the nascent relationship between pediatric rheumatology and artificial intelligence, highlighting both its advancements and constraints., (© 2024. The Author(s).)

Background/aim: Video pediatric gait, arms, leg, and spine (v-pGALS) is a virtual application of the pediatric gait, arms, leg, and spine (pGALS) examination performed by video. We aimed to verify the applicability, validity, and accuracy of the Turkish translation of v-pGALS in a large pediatric patient cohort., Materials and Methods: Children aged 4-18 years seen between May and June 2022 were included. A hands-on physical examination and v-pGALS were performed. Demographics, active symptoms, physical examination findings, diagnosis, and v-pGALS findings were recorded. The acceptability of v-pGALS, in terms of additional distress and duration, was measured by the parent/patient using a visual analog scale (VAS)., Results: 102 patients (median age 12.41 years) were included. Juvenile idiopathic arthritis (JIA) was the most common diagnosis. The median duration of v-pGALS was 7 min. An abnormal v-pGALS was identified in 25 patients while the hands-on physical examination was abnormal in 27 patients. Scoliosis and pes planus were missed in v-pGALS. Both children and parents gave a median VAS score of 0 for additional discomfort and duration. That is, the duration of v-pGALS was acceptable for ≥98% of the patients/parents, and ≥98% mentioned that it caused little/no discomfort. The sensitivity and specificity of v-pGALS were 92.6% and 100%, respectively, for the detection of musculoskeletal (MSK) abnormalities., Conclusion: The v-pGALS is an applicable, accurate, and practical tool for evaluating MSK problems in children. The Turkish translation was also conveniently acceptable., Competing Interests: Conflicts of interest: The authors declare they have no conflicts of interest associated with this study., (© TÜBİTAK.)

Eric Bywaters and Barbara Ansell were, without doubt, two of the giants in the field of Rheumatology. With their keen clinical observations and their visionary development of a dedicated multidisciplinary program focusing on diagnosis, treatment, and research, they are remembered as the founders of the modern specialty of Pediatric Rheumatology., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: General pediatricians often initially address children's musculoskeletal (MSK) issues and play a crucial role in triaging and managing patients' rheumatologic conditions. This study assessed the effectiveness of a structured curriculum in enhancing pediatric residents' knowledge, MSK examination skills, and confidence during a 4-week pediatric rheumatology rotation., Methods: Pediatric residents in their either second or third year who participated in the 4-week rheumatology rotation once across three academic years (July 2020-June 2023) were enrolled. Residents' knowledge, MSK examination skills, and confidence were assessed at pre- and post-rotation by using 25 multiple-choice questions, the Thai pediatric Gait Arms Legs Spine examination, and a questionnaire, respectively. The curriculum comprised instruction on MSK examinations, interactive lectures, case-based discussion, topic reviews, MSK radiology conference, clinical experience in rheumatology clinic and consultations, with self-guided learning with educational resources., Results: Fifty-eight pediatric residents (48 females, 10 males) with a mean age of 28.9 ± 0.8 years participated. Significant improvements were noted postrotation. Knowledge scores rose from 63.0 ± 12.2 to 79.7 ± 9.1 (mean difference 16.7 ± 10.3, p < 0.001). Similarly, MSK examination scores increased from 67.5 ± 14.4 to 93.6 ± 8.7 (mean difference 26.1 ± 14.6, p < 0.001). Residents also reported a marked increase in confidence across all evaluated areas, including history taking, MSK examination, arthrocentesis, and diagnosing and treating rheumatologic conditions (p < 0.001)., Conclusions: The 4-week structured curriculum in the pediatric rheumatology rotation significantly enhanced pediatric residents' knowledge, MSK examination skills, and confidence. These findings support the integration of pediatric rheumatology rotations into pediatric residency training programs., (© 2024. The Author(s).)

Objective: The COVID-19 pandemic has affected patient care in general. We aimed to analyze the impact of the pandemic on pediatric rheumatology practice., Methods: An online survey including 22 questions was created by the representatives of the Emerging RheumatoloGists and rEsearchers (EMERGE) group of the Pediatric Rheumatology European Society (PReS) on SurveyMonkey. The descriptive analysis of the responses was performed on SurveyMonkey., Results: Overall, 469 pediatric rheumatologists (F/M: 2.9) from 70 countries completed the survey. The practice of drug prescription is not affected by the pandemic, according to 65.3 % of the respondents, while 24.3 % and 16.5 % are prescribing biologic drugs and corticosteroids less often, respectively. Over 40 % of the respondents have seen an increased number of patients with vasculitis or chilblains during the pandemic. One-third of the respondents stated no adjustments in their clinical practice after 2.5 years of COVID-19 pandemic. The rest indicated implementing various changes, with an emphasis on incorporating telemedicine. Telemedicine constitutes ≥10 % of the clinical practice for one-third of the participants. Nonetheless, 35.5 % agree that there are still delays in patient care due to the pandemic. However, most (∼90 %) think our practice is returning to the pre-pandemic routine., Conclusion: The findings of our study indicate a significant alteration in pediatric rheumatology practice due to the pandemic. This includes increased caution when prescribing anti-rheumatic drugs, a transition towards telemedicine utilization, delays in routine care, and a rise in COVID-19-related inflammatory conditions. It is imperative to address these aspects in order to improve patient care in pediatric rheumatology., Competing Interests: Declaration of Competing Interest Gmuca S is supported by NIAMS of the National Institutes of Health under award number K23AR081409 (Gmuca). The content of the manuscript is solely the responsibility of the author and does not necessarily represent the official views of the National Institutes of Health. Other authors declare no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: Pediatric rheumatology faces a looming supply-demand crisis. While strategies have been proposed to address the supply shortfall, investigation into the increased demand for pediatric rheumatic care has been limited. Herein, we analyze new patient visits to a large tertiary care pediatric rheumatology center to identify emerging trends in referrals and areas for potential intervention to meet this increased demand., Methods: All patients referred to and seen by the University of Alabama at Birmingham Pediatric Rheumatology Division between January 2019 and December 2021 for a new patient evaluation were identified. Patient data was retrospectively abstracted, de-identified, and analyzed to develop trends in referrals and frequency of rheumatic disease, non-rheumatic disease, and specific diagnoses., Results: During the study period, 2638 patients were referred to and seen in by the pediatric rheumatology division. Six hundred and ten patients (23.1%) were diagnosed with rheumatic disease. The most common rheumatic disease was juvenile idiopathic arthritis (JIA) at 45.6%, followed by primary Raynaud phenomenon (7.4%), recurrent fever syndromes (6.9%), vasculitides (6.7%), and inflammatory eye disease (6.2%). Of the 2028 patients (76.9%) diagnosed with a non-rheumatic condition, benign musculoskeletal pain was the most common (61.8%), followed by a combination of somatic conditions (11.6%), and non-inflammatory rash (7.7%)., Conclusion: In this analysis of new patient referrals to a large pediatric rheumatology center, the majority of patients were diagnosed with a non-rheumatic condition. As a worsening supply-demand gap threatens the field of pediatric rheumatology, increased emphasis should be placed on reducing non-rheumatic disease referrals., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Objectives: The rapid expansion in the use of telemedicine after the COVID-19 pandemic has led many patients with chronic diseases to seek alternative ways for follow-ups. This study aimed to investigate the demands and opinions of parents of children with rheumatic diseases toward telemedicine and to examine the factors affecting telemedicine preference. Methods: A single-center, cross-sectional, Web-based survey study was conducted. Sociodemographic data, characteristics of the disease, access to the clinic, internet use, and views on telemedicine were assessed. Factors effecting telemedicine preference were evaluated by multivariate analysis. Results: A total of 245 parents have completed the survey. The diagnoses of patients were recurrent fever syndromes (55.1%), juvenile idiopathic arthritis (31.0%), systemic connective tissue diseases (8.2%), and vasculitis (5.7%). The majority of patients came to the clinic by public transport ( n  = 190, 77.6%). Sixty-eight (27.8%) patients missed at least one outpatient appointment in the last year. Majority ( n  = 172, 70.2%) of parents stated that they would prefer telemedicine visits if it becomes available. Multivariate analysis revealed that the most related factors to telemedicine preference were higher education level (odds ratio [OR]: 6.69, confidence interval [95% CI]: 2.21-20.25, p  = 0.001), missing an appointment (OR: 3.04, 95% CI: 1.41-6.56, p  = 0.004), and travel time longer than 1 h (OR: 2.13, 95% CI: 1.13-3.86, p  = 0.012). Conclusion: Telemedicine visits are in demand in pediatric rheumatology and should be considered an alternative method to ensure continuity of patient follow-up. A personal approach should be followed when selecting patients for telemedicine.

Background: Given the impact of psychological factors on rheumatic disease, pediatric psychologists serve a vital role in promoting quality of life and managing common problems among youth with rheumatic disease. The aim of this project was to increase access to psychological services among youth with rheumatic disease at a children's hospital., Methods: A quality improvement (QI) team identified key drivers and interventions aimed to increase access to psychological services for youth with rheumatic disease. Data was collected for a 6-month baseline period and 4-year intervention period. We applied the Plan-Do-Study Act method of QI and the American Society for Quality criteria to adjust the center line and control limits., Results: There were two statistically significant center line shifts in the number of patients seen by psychology and one statistically significant shift in referrals to psychology over time with applied stepwise interventions. Patients seen by a psychologist increased by 3,173% from a baseline average of 1.8 to 59.9 patients seen per month (p < 0.03). Psychology referrals increased by 48% from a baseline average of 9.85 to 14.58 referrals per month over the intervention period (p < .01)., Conclusions: Youth with rheumatic disease received increased access to mental health treatment when psychological services were imbedded within rheumatology care. Psychology referrals also increased significantly, suggesting that psychology integration within a medical clinic can increase identification of needs. Results suggest that psychology integration into rheumatology care may increase access to mental health treatment and identification of psychological needs in this at-risk population., (© 2023. The Author(s).)

Background: Diversity management has gained traction in Japan. The Pediatric Rheumatology Association of Japan (PRAJ) has an Advisory Committee for Diversity Promotion with a broader focus on promoting diversity. The objectives of this study were to better understand the problems faced by PRAJ members regarding the work environment, childcare and nursing care, and work-life balance., Methods: A web-based questionnaire was administered to members of the PRAJ and 79 responses were evaluated., Results: Of the respondents, 73% were male and 27% were female. A total of 14% worked for more than 12 h on weekdays, and 22% worked for more than 60 h per week and 38% had fewer than 4 days off per month. Regarding childcare, 54% of the respondents were raising preschool children and 83% had taken parental leave for less than 1 year. A total of 17% of participants had family members in need of care. For both childcare and caregiving, the burden was greater for women. Only 18% of the respondents reported a well-balanced work-life balance, and the most common reasons for a lack of balance were not having enough time, heavy workload, and heavy housework load., Conclusions: The working hours of the respondents were long, and female members had a greater burden of childcare and caregiving, which was considered a barrier to the career development of women. In the future, there will be a need to promote a sense of equality in diverse human resources, develop support for family life, and shorten working hours., (© 2023 Japan Pediatric Society.)

Background: Children with juvenile idiopathic arthritis (JIA) who achieve a drug free remission often experience a flare of their disease requiring either intraarticular steroids (IAS) or systemic treatment with disease modifying anti-rheumatic drugs (DMARDs). IAS offer an opportunity to recapture disease control and avoid exposure to side effects from systemic immunosuppression. We examined a cohort of patients treated with IAS after drug free remission and report the probability of restarting systemic treatment within 12 months., Methods: We analyzed a cohort of patients from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry who received IAS for a flare after a period of drug free remission. Historical factors and clinical characteristics and of the patients including data obtained at the time of treatment were analyzed., Results: We identified 46 patients who met the inclusion criteria. Of those with follow up data available 49% had restarted systemic treatment 6 months after IAS injection and 70% had restarted systemic treatment at 12 months. The proportion of patients with prior use of a biologic DMARD was the only factor that differed between patients who restarted systemic treatment those who did not, both at 6 months (79% vs 35%, p < 0.01) and 12 months (81% vs 33%, p < 0.05)., Conclusion: While IAS are an option for all patients who flare after drug free remission, it may not prevent the need to restart systemic treatment. Prior use of a biologic DMARD may predict lack of success for IAS. Those who previously received methotrexate only, on the other hand, are excellent candidates for IAS., (© 2022. The Author(s).)

Objective: To investigate the relationship between age and symptom duration at initial presentation to pediatric rheumatology for juvenile idiopathic arthritis (JIA)., Methods: In children and young people (CYP) enrolled in the Childhood Arthritis Prospective Study prior to March 2018, an association between age at presentation (&lt; 5, 5-11, and &gt; 11 yrs) and symptom duration was tested by multivariable linear regression., Results: In 1577 CYP, 5- to 11-year-olds took 3.2 months longer and &gt; 11-year-olds 6.9 months longer to reach pediatric rheumatology than &lt; 5-year-olds., Conclusion: Adolescents take longer to reach pediatric rheumatology, potentially affecting their longer-term outcomes given the window of opportunity for JIA treatment., (Copyright © 2022 by the Journal of Rheumatology.)

Background: To report baseline characteristics, patient reported outcomes and treatment of children with Juvenile Dermatomyositis (JDM) in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry., Methods: Children newly diagnosed with JDM were enrolled in the CARRA Registry from 41 pediatric rheumatology centers. Baseline patient demographics, disease characteristics, assessments, patient reported outcome and treatments were recorded., Results: In the first year, 119 JDM participants were enrolled. Most were female (63.4%), and white (72.3%) with a median diagnosis age 8.0 years (IQR 4.0-11.5), and median age of disease onset 7.0 years (IQR 3.5-7.5). They had characteristic rashes (92.4%), elevated muscle enzymes (83.2%), physician global score 4.0 (IQR 2.5-5.0) and manual muscle testing score 63.5 (IQR 51.0-75.0). Calcinosis (3.4%) and interstitial lung disease (< 1%) were uncommon. Myositis specific antibodies were measured and reported in nearly half of participants enrolled where anti-MJ followed by Anti-p155/140 were most common (11/49 and 7/53 respectively). Childhood Health Assessment Questionnaire (CHAQ) results showed mild-moderate disability (median 0.750, IQR 0.030-1.875), as did patient/parent global assessments of disease activity (median 3, patient IQR: 1.75-5.25; parent IQR: 1-7). Patient Reported Outcomes Measurement Information System (PROMIS®) Pediatric Global Health 7 scores, Pain Interference, Physical Function scores for Mobility, and Upper Extremity Function were commonly worse than 95% of the general pediatric population., Conclusions: In its inaugural year, 119 JDM patients were successfully enrolled in participapte in the New CARRA Registy. This registry will provide the necessary foundation to advance clinical research to improve outcomes using traditional measures and patient reported outcomes. With the CARRA biorepository, this infrastructure will enable future translational research. Together, these efforts may aid in future clinical trials, including comparative effectiveness trials., (© 2022. The Author(s).)

Implementation science is the study of processes that promote reliable uptake of evidence-based practices into clinical care. The integration of implementation science and health disparities research approaches has been proposed as a method to reduce health inequity through detection, understanding, and implementation of health equity-focused interventions. In this review, we provide an argument for the study of implementation science in pediatric rheumatology in light of previously observed health disparities, present a framework for the study of health equity and implementation science in pediatric rheumatology, and propose next steps to accelerate action., Competing Interests: Disclosure E.A. Smitherman is supported by grant funding from the Lupus Foundation of America and Childhood Arthritis and Rheumatology Research Alliance. E. von Scheven is supported by the Childhood Arthritis and Rheumatology Research Alliance and grant funding from the Patient-Centered Outcomes Research Institute. The authors have no commercial or financial conflicts to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Both pediatric and adult rheumatology care teams play a central role in health care transition, the shift from child- and family-centered to adult-oriented health care. Components of transition preparation include readiness assessment, setting self-management goals, and spending time in clinical visits without a parent present. Pediatric providers and families should work together to create a transfer plan, identifying a new adult rheumatology care provider, providing a medical summary before transfer, and anticipating changes in health insurance. For high-risk transfers, direct communication between providers is recommended. Finally, adult rheumatologists need to build rapport with young adults to support future engagement in care., Competing Interests: Disclosure Dr R.E. Sadun is the recipient of a Clinician Scholar Educator Award from the Rheumatology Research Foundation to complete a project that entails developing a transition curriculum for rheumatology fellows., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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.)

The electronic health record (EHR) ecosystem is undergoing rapid evolution in response to new rules and regulations promulgated by the US HITECH Act (2009) and the 21st Century Cures Act (2016), which together promote and support enhanced information use, access, exchange, as well as vendor-agnostic application development. By leveraging emerging new standards and technology for EHR data interchange, for example, FHIR and SMART, pediatric rheumatology clinical care, research, and quality improvement communities will have the opportunity to streamline documentation workflows, integrate patient-reported outcomes into clinical care, reuse clinical data for research purposes, and embed implementation science approaches within the EHR., Competing Interests: Funding Dr. A.J. Taxter receives salary support from CARRA as her role as the CARRA Informatics Associate. Disclosure Dr A.J. Taxter is a volunteer member of the Epic Rheumatology Steering Board. Dr M.D. Natter has nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Mental health problems are more common in children with pediatric rheumatologic diseases (PRDs) than healthy peers. Mental health problems affect disease-related outcomes and health-related quality of life (HRQOL), so addressing these problems can improve clinical and psychosocial outcomes. Mental health screening tools are available, and there are resources available to aid in integrating mental health care into the clinical setting. By implementing these tools, mental health problems can be recognized and addressed., Competing Interests: Disclosure Dr E.B. Treemarcki is currently the recipient of a Research Grant from the Childhood Arthritis and Rheumatology Research Alliance and the Arthritis Foundation (CARRA-AF). Dr N.R. Cunningham is currently the recipient of a training grant from NIH/NCCIH (K23 AT009458) and a Transdisciplinary Research Grant from the Childhood Arthritis and Rheumatology Research Alliance and the Arthritis Foundation (CARRA-AF). Dr A.M Knight and Dr A.N. Danguecan have nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

This article provides an in-depth review of telemedicine and its use in pediatric rheumatology. Historical barriers to the use of telemedicine in pediatric chronic care are described, and recent policy changes that have supported the use of telemedicine are discussed. Future directions and suggestions for the evaluation of telemedicine in pediatric rheumatology care are provided with a special focus on clinical outcomes, its use in research, patient acceptability, and health equity., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

The advent of social media has revolutionized and empowered communities of people living with rare diseases. Social media has enabled families of children with similar pediatric rheumatic diseases (PRDs) to meet regardless of geographic distance, allowing them to support each other and join together to advocate on behalf of their children. Researchers have also leveraged social media to learn about the health of patients and their communities. This article reviews the promises and perils of social media related to health, focusing on its potential use to support research and care of children with PRD., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Pediatric rheumatology subspecialists treat chronic autoimmune diseases with onset in childhood. Prompt diagnosis and ongoing management of these conditions are imperative to prevent damage from ongoing inflammation. Here, we aim to describe the current landscape of pediatric rheumatic disease in lower to middle-income countries (LMICs) and explore current barriers to understanding global disease burden. We then examine innovative strategies to promote a more equitable future for children and young people living with rheumatic diseases worldwide., Competing Interests: Disclosure Drs L.B. Lewandowski, S. Sawhney, and C. Scott have no financial disclosures or conflicts of interest to declare. The opinions expressed in this article are the author's own and do not reflect the view of the National Institutes of Health, the Department of Health and Human Services, or the United States government., (Published by Elsevier Inc.)

Background: During the Coronavirus disease 2019 pandemic, ambulatory pediatric rheumatology healthcare rapidly transformed to a mainly telehealth model. However, pediatric patient and caregiver satisfaction with broadly deployed telehealth programs remains largely unknown. This study aimed to evaluate patient/caregiver satisfaction with telehealth and identify the factors associated with satisfaction in a generalizable sample of pediatric rheumatology patients., Methods: Patients with an initial telehealth video visit with a rheumatology provider between April and June 2020 were eligible. All patients/caregivers were sent a post-visit survey to assess a modified version of the Telehealth Usability Questionnaire (TUQ) and demographic and clinical characteristics. TUQ total and sub-scale (usefulness, ease of use, effectiveness, satisfaction) scores were calculated and classified as "positive" based on responses of "agree" or "strongly agree" on a 5-point Likert scale. Results were analyzed using standard descriptive statistics and Wilcoxon signed rank testing. The association between demographic and clinical characteristics with TUQ scores was assessed using univariate linear regression., Results: 597 patients/caregivers met inclusion criteria, and the survey response rate was 42% (n = 248). Juvenile idiopathic arthritis was the most common diagnosis (33.5%). The majority of patients were diagnosed greater than 6 months previously (72.6%) and were prescribed chronic medications (59.7%). The median total TUQ score was 4 (IQR: 4-5) with positive responses in 81% of items. Of the subscales, usefulness scores were lowest (median: 4, p < 0.001). Telehealth saves time traveling was the highest median item score (median = 5, IQR: 4-5). Within subscales, items that scored significantly lower included convenience, providing for needs, seeing rheumatologist as well as in person, and being an acceptable way to receive rheumatology services (all p < 0.001). There were no significant demographic or clinical features associated with TUQ scores., Conclusions: Our results suggest telehealth is a promising mode of healthcare delivery for pediatric rheumatic diseases but also identifies opportunities for improvement. Innovation and research are needed to design a telehealth system that delivers high quality and safe care that improves healthcare outcomes. Since telehealth is a rapidly emerging form of pediatric rheumatology care, improved engagement and training of patients, caregivers, and providers may help improve the patient experience in the future., (© 2021. The Author(s).)

Background: Pediatric rheumatology (PR) is a relatively new and rare subspecialty in emerging countries. This study aimed to investigate physicians' attitudes toward and real-life clinical practice in PR among residency-trained pediatricians in Thailand., Methods: An electronic questionnaire was developed and sent via email to pediatricians from Thailand who completed their residency training between 2007 and 2015. Confidence in treating and managing children with rheumatic diseases was rated using a 5-point Likert scale., Results: The response rate was 281 out of 902 (31%), and the mean ± standard deviation age of respondents was 33.8 ± 2.7 years. Confidence was rated as adequate for history taking of children with rheumatic diseases (mean 2.76, 95% confidence interval [CI]: 2.66-2.91), but low for musculoskeletal (MSK) examination (2.42, 95% CI: 2.29-2.54), arthrocentesis (2.01, 95% CI: 1.91-2.11), and rheumatology investigation (2.49, 95% CI: 2.39-2.60). Confidence was high for diagnosis and management of Henoch-Schönlein purpura (3.83, 95% CI: 3.74-3.92) and Kawasaki disease (3.46, 95% CI: 3.38-3.55), but low for juvenile idiopathic arthritis (JIA) (2.41, 95% CI: 2.30-2.52) and chronic systemic vasculitis (1.97, 95% CI: 1.85-2.08). Confidence to perform an MSK examination and arthrocentesis was significantly higher in respondents who had a full-time pediatric rheumatologist working in their pediatric residency training center (P = 0.02, P = 0.01, respectively), and in those who had experienced a PR rotation (P < 0.001, P = 0.01, respectively). Most respondents agreed that more teaching in PR is essential (95.3%) and that case-based discussion was the preferred teaching method., Conclusion: The self-rated confidence of pediatricians was low in MSK examination, arthrocentesis, and rheumatology investigation and therefore, teaching on PR is needed., (© 2021 Japan Pediatric Society.)

Juvenile systemic sclerosis (jSSc) is a rare, severe autoimmune disease associated with life-threatening multiorgan inflammatory-driven fibrosis. Recognition early in the disease process, when treatment is more effective, is critical. We outline insights from the authors, who specialize and host jSSc cohorts, combined with recent literature review combining available juvenile-onset and applicable adult-onset studies regarding SSc evaluation, which can be extrapolated to children. Practice tips are provided for each main organ system., Competing Interests: Declaration of competing interest The authors have nothing to disclose., (Copyright © 2021. Published by Elsevier Ltd.)

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

Data on therapy of COVID-19 in immunocompetent and immunosuppressed children are scarce. We aimed to explore management strategies of pediatric rheumatologists. All subscribers to international Pediatric Rheumatology Bulletin Board were invited to take part in an online survey on therapeutic approaches to COVID-19 in healthy children and children with autoimmune/inflammatory diseases (AID). Off-label therapies would be considered by 90.3% of the 93 participating respondents. In stable patients with COVID-19 on oxygen supply (stage I), use of remdesivir (48.3%), azithromycin (26.6%), oral corticosteroids (25.4%) and/or hydroxychloroquine (21.9%) would be recommended. In case of early signs of "cytokine storm" (stage II) or in critically ill patients (stage III) (a) anakinra (79.5% stage II; 83.6% stage III) or tocilizumab (58.0% and 87.0%, respectively); (b) corticosteroids (oral 67.2% stage II, intravenously 81.7% stage III); (c) intravenous immunoglobulins (both stages 56.5%); or (d) remdesivir (both stages 46.7%) were considered. In AID, > 94.2% of the respondents would not support a preventive adaptation of the immunomodulating therapy. In case of mild COVID-19, more than 50% of the respondents would continue pre-existing treatment with immunoglobulins (100%), hydroxychloroquine (94.2%), anakinra (79.2%) or canakinumab (72.5%), or tocilizumab (69.8%). Long-term corticosteroids would be reduced by 26.9% (< = 2 mg/kg/d) and 50.0% (> 2 mg/kg/day), respectively, with only 5.8% of respondents voting to discontinue the therapy. Conversely, more than 75% of respondents would refrain from administering cyclophosphamide and anti-CD20-antibodies. As evidence on management of pediatric COVID-19 is incomplete, continuous and critical expert opinion and knowledge exchange is helpful.

There is no single diagnostic test for any rheumatic disease. The diagnosis of a rheumatic disease is made by the sum of the findings in history, physical examination, laboratory, and imaging tests. A differential diagnosis list in pediatric rheumatology is quite long and mainly includes malignant, infectious, and inherited metabolic disorders. We aim to present cases that were referred to a pediatric rheumatology outpatient clinic with provisional diagnosis of a rheumatic disease but finally diagnosed with a non-rheumatic disease in order to emphasize the importance of differential diagnoses. Eight cases were presented in this manuscript. Five cases were referred with the provisional diagnosis of juvenile idiopathic arthritis. Sarcoidosis, chronic non-bacterial osteomyelitis, and autoinflammatory disease were the provisional referral diagnoses in three patients. Definitive diagnoses of the patients were as follows: acute lymphoblastic leukemia (two cases), bilineage acute leukemia, Hodgkin lymphoma, brucellosis, mucolipidosis type III, anhidrotic ectodermal dysplasia, and Freiberg disease. In children presenting with rheumatic complaints malignant, infectious and inherited metabolic disorders should always be in the differential diagnosis list of a pediatric rheumatologist. Alternative diagnoses should always be considered even in patients with a rheumatic disease when the patient does not respond to treatment or follows an unusual clinical course. Key Points • Diagnosis of a rheumatic disease is made by exclusion of all other pathologies. • Malignant and infectious diseases may mimic the signs and symptoms of a rheumatic disease.

Objectives: To determine the feasibility of comparing the Childhood Arthritis and Rheumatology ResearchAlliance (CARRA) consensus treatment plans (CTP) in treating moderate new-onset juvenile dermatomyositis (JDM) using the CARRA registry, and to establish appropriate analytic methods to control for confounding by indication and missing data., Methods: A pilot cohort of 39 patients with JDM from the CARRA registry was studied. Patients were assigned by the treating physician, considering patient/family preferences, to 1 of 3 CTP: methotrexate (MTX) and prednisone (MP); intravenous (IV) methylprednisolone, MTX, and prednisone (MMP); or IV methylprednisolone, MTX, prednisone, and IV immunoglobulin (MMPI). The primary outcome was the proportion of patients achieving moderate improvement at 6 months under each CTP. Statistical methods including multiple imputation and inverse probability of treatment weighting were used to handle missing data and confounding by indication., Results: Patients received MP (n = 13), MMP (n = 18) and MMPI (n = 8). Patients in all CTP had significant improvement in disease activity. Of the 36 patients who remained in our pilot study at 6 months, 16 (44%) of them successfully achieved moderate improvement at 6 months (6/13, 46% for MP; 7/15, 47% for MMP; 3/8, 38% for MMPI). After correcting for confounding, there were no statistically significant pairwise differences between the CTP ( P = 0.328-0.88)., Conclusion: We gained valuable experience and insight from our pilot study that can be used to guide the design and analysis of comparative effectiveness studies using the CARRA registry CTP approach. Our analytical methods can be adopted for future comparative effectiveness studies and applied to other rare disease observational studies.

Objectives: The COVID-19 pandemic is a global health problem. We, as the EMERGE (EMErging RheumatoloGists and rEsearchers) group of PReS (Pediatric Rheumatology European Society) analyzed how the pandemic has affected pediatric rheumatology practice., Methods: An online survey was developed to assess changes in pediatric rheumatology practice due to the pandemic. Results were analyzed using descriptive statistics., Results: From 70 countries, 493 pediatric rheumatologists (80.3% in pediatric rheumatology practice for ≥5 years) responded to the survey. Around 70% disagreed that the pandemic led to reduced prescription of nonsteroidal anti-inflammatory drugs, conventional synthetic and biologic disease-modifying antirheumatic drugs. Almost half were more likely to taper corticosteroids faster. One-fifth hesitated to switch the major immunosuppressant during a flare. Patients encountering difficulties obtaining hydroxychloroquine and tocilizumab due to shortages were noted by 192 (38.9%) and 44 (8.9%), respectively. Twenty to 30% indicated that their patients had experienced a flare or delay in diagnosis/intervention due to postponed appointments.53% mentioned use of phone calls/smartphone applications while 47% shifted towards video consultations for patient care. Respondents indicated an increased number of patients with Kawasaki disease (30%), macrophage activation syndrome (15.6%), unusual vasculitic rashes (31.4%), and hyperinflammation (33.5%) during the pandemic., Conclusion: This is the largest survey to date addressing changes in pediatric rheumatology practice due to the COVID-19 pandemic. Primary changes were due to delays in clinic appointments, increase in use of virtual technologies, and concerns about the use of immunosuppressive therapies. An increased number of patients with Kawasaki disease/hyperinflammation mentioned by the respondents is noteworthy., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The role of ultrasound imaging in the diagnosis and monitoring of paediatric rheumatic diseases with special emphasis on recent scientific work regarding the evidence base and standardization of this technique is being reviewed. An overview of the most important practical aspects for the use of musculoskeletal ultrasound in a clinical setting is also provided. Huge scientific efforts and advances in recent years illustrate the increasing importance of musculoskeletal ultrasound in pediatric rheumatology. Several studies focused on setting an evidence-based standard for the ultrasound appearance of healthy and normal joints in children of all age groups. Physiologic vascularization and ossification were two main aspects of these studies. Other publications demonstrate that ultrasound imaging is also an important and useful tool to detect pathology as synovitis, tenosynovitis or enthesitis in children and to monitor pediatric patients with rheumatic conditions. Important practical aspects include training in the use of correct ultrasound techniques, as well as knowledge and experience of normal pediatric sonoanatomy and the appearance of pathological findings on ultrasound., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Parenteral treatments (either subcutaneous or intravenous) are frequently used in rheumatology practice. In this study, drug side effects in patients who were followed up with a rheumatic disease and treated with parenteral administration methods were evaluated. The drug side effects in children who were followed up with a rheumatic disease and treated with parenteral treatments between 2010 and 2019 were recorded, retrospectively. All parenteral treatments are applied by a clinical nurse specialist (CNS) who is experienced in pediatric rheumatology for 10 years. Four hundred and thirteen patients were evaluated in this study. The mean age was 12.09 ± 5.05 years. Most of them were diagnosed with juvenile idiopathic arthritis (n = 317) and colchicine-resistant familial Mediterranean fever (n = 57). Among the patients, 287 was treated with methotrexate, 130 with etanercept, 90 with adalimumab, 71 with anakinra, 64 with canakinumab, 55 with tocilizumab, seven with rituximab, six with infliximab, and four with abatacept. Two of the patients had a history of drug allergy (ceftriaxone = 1, ranitidine = 1). The most common adverse reactions were as follows: nausea-vomiting in 52, rash in 11, itching in three, chest tightening in two, bruising in two, headache in two, and abdominal pain in one of the patients. Drug side effects were observed after an average of three (1-4) administrations. Antihistaminic and steroids (tocilizumab = 3, infliximab = 1, methotrexate = 1) were administered to five patients due to hypersensitivity reactions. Considering the possible side effects and preparation protocols of parenteral treatments, experienced physicians and nurses are required in the field.

To describe the demographic characteristics and clinical features of patients referred to a pediatric rheumatology outpatient clinic in Turkey and to compare the final diagnoses with the previous literature data. All new patients referred to pediatric rheumatology outpatient clinic of Kanuni Sultan Süleyman Research and Training Hospital between March 2018 and March 2019 were enrolled to the study. Demographic data, referral patterns, disease related features, physical examination findings and final diagnoses of new referrals were collected prospectively. A total of 2982 new referrals were evaluated in 1-year period. Among them 1561 (52%) had a diagnosis of a rheumatic disease. The frequencies of most common rheumatic diseases were; periodic fever syndromes (47.3%), juvenile idiopathic arthritis (18%) and vasculitis (14.4%), respectively. Non-rheumatic conditions were diagnosed in 1243 patients, among them orthopedic/mechanic problems (27.4%) were the most frequent ones followed by vitamin D deficiency (17.5%) and dermatological problems (9.8%). Patients with non-rheumatic conditions comprised a large part of the pediatric rheumatology outpatient clinic. National registries are required to establish the frequencies of pediatric rheumatic diseases in Turkey.

Pediatric Rheumatology is an emerging specialty in Turkey with increasing number of available centers and specialists all over the country. In this paper, we sought to provide an overview on pediatric rheumatology service in our country, as well as to assess the principle published literature from Turkey in this field. A systematic literature search has been performed to achieve the significance and the impact of this manuscript. The most relevant used databases (PubMed/MEDLINE, Web of Science, SCOPUS) for peer-reviewed studies and reviews in English language published during the last 5 years were screened. In the first part of the manuscript, we tried to give more details on the history of pediatric rheumatology in Turkey. In further text, we put an accent over the most common rheumatologic conditions among children in Turkey, including Familial Mediterranean fever, juvenile idiopathic arthritis, juvenile spondyloarthropathies, and childhood vasculitides. Despite the considerable literature from Turkey on pediatric rheumatic diseases, a need for unique strategies that would guide the management of rheumatic diseases in childhood remains open. The cultural and historical inheritance together with geographical position make the Turkey a suitable ground for investigations in filed of auto-inflammation and all other inflammatory conditions. Prospective, multicentric studies especially among rheumatologic conditions common in this part of the world would give us more relevant data and open new horizons in diseases' management. International collaborations and databases should be highly encouraged and supported, to make the care of pediatric rheumatic disease uniform.

Pediatric rheumatology is an exciting and rewarding career area. However, challenges when attracting trainees to this field include practice often occurring in smaller groups compared with general pediatrics, available positions requiring relocation, and fluctuation in funding resulting in uncertainty regarding training positions. Having critical mass in pediatric divisions is important to ensure adequate mentoring and people power to produce scholarly work, reduce on-call frequency and mitigate faculty absences that result in unplanned addition of clinical work. Compensation has historically lagged behind that of general pediatrics. Increased research opportunities through organized networks, patient and parent engagement, and the increasing recognition of pediatric rheumatologists as contributing to scholarship has heightened the profile of pediatric rheumatology nationally and internationally., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Pediatric rheumatologic disease occurs more frequently than several other chronic pediatric diseases but is often underrecognized. It is estimated that in the US, one in 250 children has some form of juvenile arthritis and 300,000 children have a form of rheumatologic disease. However, there are only approximately 400 practicing pediatric rheumatologists nationwide., Methods: Kern's six-step method was used to develop a pediatric rheumatology curriculum based on respondents' perceived lack of training and comfort with four key areas: workup, musculoskeletal exam, laboratory interpretation, and referral to rheumatology. These cases were developed for second-year pediatric and second- and third-year internal medicine-pediatric residents rotating with the service. The curriculum was composed of four 30-minute case discussions as well as an observed musculoskeletal exam session., Results: In 2017, weekly case study sessions reached 34 trainees. Survey results from these trainees are representative of our overall results and reveal that learners felt the content of the cases helped increase comfort with compiling pertinent history and information of symptoms consistent with autoimmune disease, recognizing physical exam findings of autoimmune disease, ordering and interpreting laboratory studies in children with concerns for autoimmune disease, and referring to pediatric rheumatology., Discussion: This case-based curriculum exposed residents to presentations of the more common autoimmune diseases encountered in the pediatric population. The curriculum helps fill a gap in pediatric training through increased exposure to this subset of chronic diseases and expands physical examination skills not typically taught in general pediatrics., Competing Interests: None to report.

Background: The United States pediatric rheumatology workforce is committed to a mission of providing children access to pediatric rheumatology care. With a limited number and distribution of pediatric rheumatologists, telemedicine has been proposed as one way to meet this mission, yet the adoption of this modality has been slower than expected. The purpose of this study was to explore the parent perspective on barriers to accessing pediatric rheumatology care and to explore the acceptability of telemedicine and other alternative care models., Methods: Over a period of six weeks, all new and return English-speaking parents/guardians of patients visiting a single center were offered an opportunity to complete a survey which assessed barriers to care and interest in alternative models of care. Responses were analyzed using descriptive statistics., Results: Survey response rate was 72% (159/221). Twenty-eight percent (45/159) traveled more than three hours to the pediatric rheumatology clinic, and 43% (65/152) reported travel as inconvenient. An overwhelming majority of respondents (95%, 144/152) reported a preference for in-person visits over the option of telemedicine. This preference was similar regardless of whether respondents reported travel to the clinic as inconvenient vs convenient (inconvenient 92%, 60/65; convenient 97%, 84/87; p = 0.2881) and despite those reporting travel as inconvenient also reporting greater difficulty with several barriers to care. Those familiar with telemedicine were more likely to report a preference for telemedicine over in-person visits (27%, 3/11 vs 3%, 4/140; p = 0.0087). The option of an outreach clinic was acceptable to a majority (63%, 97/154); however, adult rheumatology and shared-care options were less acceptable (22%, 35/156 and 34%, 53/156 respectively)., Conclusion: Among survey respondents, in-person visits were preferred over the option of telemedicine, even when travel was noted to be inconvenient. Telemedicine familiarity increased its acceptability. Outreach clinics were acceptable to a majority. Ultimately, the parent perspective can shape acceptable ways to address barriers and provide accessible care.

Colombia is a densely populated country with a small number of pediatric rheumatology specialists, including 14 specialists for a population of 1,927,000 children in 2014. The objective of the study was to improve the skills required for early identification, timely referral, and management of musculoskeletal diseases, especially juvenile idiopathic arthritis (JIA), in a group of pediatricians and pediatric residents in a remote region of Colombia. Supported by grant programs developed by the International League of Associations for Rheumatology (ILAR), a combined educational strategy (blended learning) was implemented based on two classroom educational activities and four online modules. The students' acquired knowledge and perception of the strategy were evaluated. Scores were reported as median values and interquartile ranges (IQR), and the differences between scores were estimated using the Wilcoxon test for equal medians. Forty-one students were enrolled, 37 completed the online modules, and 33 attended the final in-person session. The results of the written tests demonstrated an improved ability to solve clinical problems compared with the results of the tests before the course (the median initial vs. final test scores 3 (IQR = 1) vs. 5 (IQR = 0), p = 0.000). The students reported high levels of satisfaction related to compliance with the proposed objectives, the relevance of the contents and activities performed, and the impact on everyday practice. These types of strategies are useful as tools for continuing medical education. However, the results pertain only to short-term learning. It is necessary to evaluate their impact on "lifelong learning."

Background: This study looks at access to care for Juvenile Idiopathic Arthritis through pediatric rheumatology in the UAE, as an example of multi-ethnic society., Methods: Patients with a diagnosis of Juvenile idiopathic arthritis were identified through the hospital electronic medical records system from January 1st 2011 to December 31st 2014. All residents of the United Arab Emirates hold an Emirates identity card. We divided our patients into two groups: Emirati-Emirates, who are native Emirati children and hold the Emirati nationality, as stated on their Emirates identity card, and who therefore have full, comprehensive access to free medical care; and non-Emirati-Emirates, who represent other nationalities, as stated on their Emirates identity card. The primary objective of this study is to look at access to care for Juvenile idiopathic arthritis through pediatric rheumatology in the two groups. The secondary objective is to look at the effect of having multiple types of healthcare insurance coverage on access to biologics. A retrospective review was carried out., Results: Sixty-six patients with JIA identified: 33 Emirates and 33 non-Emirates. For Emirates, the mean time from onset to first appointment with pediatric rheumatologist and diagnosis is 9 months (range: 1-48), and for non-Emirates is 12.4 months (range: 1-96). Among the Emirates, 10 patients are currently on biologic with methotrexate. Among the non-Emirates, 15 are on biologic with methotrexate. Among the Emirates, 12 are currently in remission while on treatment, as are 10 non-Emirates. Regarding disability, one Emirati patient has blindness secondary to noncompliance while under previous treatment. One Non-Emirati developed joint deformities due to periods of noncompliance and no follow up., Conclusions: Delay in presentation to pediatric rheumatology has been identified as an important factor in our population, which is multi-cultural and multi-ethnic. Type of health care insurance cover did not affect number of patients getting biological therapy once patient seen in the pediatric rheumatology service.

Objective: To identify targets for improving mental healthcare of adolescents with systemic lupus erythematosus (SLE) by assessing current practices and perceived barriers for mental health intervention by pediatric rheumatology clinicians., Methods: Members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) completed a Web-based survey assessing current mental health practices, beliefs, and barriers. We examined associations between provider characteristics and the frequency of barriers to mental health screening and treatment using multivariable linear regression., Results: Of the 375 eligible CARRA members, 130 responded (35%) and 119 completed the survey. Fifty-two percent described identification of depression/anxiety in adolescents with SLE at their practice as inadequate, and 45% described treatment as inadequate. Seventy-seven percent stated that routine screening for depression/anxiety in pediatric rheumatology should be conducted, but only 2% routinely used a standardized instrument. Limited staff resources and time were the most frequent barriers to screening. Respondents with formal postgraduate mental health training, experience treating young adults, and practicing at sites with very accessible mental health staff, in urban locations, and in Canada reported fewer barriers to screening. Long waitlists and limited availability of mental health providers were the most frequent barriers to treatment. Male clinicians and those practicing in the Midwest and Canada reported fewer barriers to treatment., Conclusion: Pediatric rheumatology clinicians perceive a need for improved mental healthcare of adolescents with SLE. Potential strategies to overcome barriers include enhanced mental health training for pediatric rheumatologists, standardized rheumatology-based mental health practices, and better integration of medical and mental health services.

To develop recommendations on the transition from pediatric care to adult care in patients with chronic inflammatory rheumatic diseases with childhood onset based. Recommendations were generated following nominal group methodology and Delphi technique. A panel of 16 experts was established. A systematic literature review (on transitional care) and a narrative review were performed and presented to the panel in the first panel meeting to be discussed. A first draft of recommendations was generated and circulated. Focal groups with adolescents, young adults and parents were organized. In a second meeting, the focus group results along with the input from invited psychologist were used to establish definitive recommendations. Then, a Delphi process (two rounds) was carried out. A group of 72 pediatric and adult rheumatologists took part. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70 % voted ≥7. The level of evidence and grade or recommendation was assessed using the Oxford center for evidence-based medicine levels of evidence. Transition care was defined as a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic inflammatory rheumatic diseases with childhood onset as they move from child-centered to adult-oriented healthcare systems. The consensus covers: transition needs, barriers and facilitators, transitional issues (objectives, participants, content, phases, timing, plans, documentation and responsibilities), physicians' and other health professionals' knowledge and skill requirements, models/programs, and strategies and guideline for implementation. Preliminary recommendations and agreement grade are shown in the Table (first Delphi round). These recommendations are intended to provide health professionals, patients, families and other stakeholders with a consensus on the transition process from pediatric to adult care.

The antinuclear antibody (ANA) test is frequently used for the identification of patients who are at a high risk of developing autoimmune rheumatological diseases. The aim of this study is to evaluate the final diagnoses of patients applied to the pediatric rheumatology outpatient clinic with a positive ANA test result. In this study, the medical records of 283 children who had ANA positivity between January 2010 and January 2022 were evaluated retrospectively. All patients were younger than 18 years of age at diagnosis and were followed up in the pediatric rheumatology department for at least 6 months. The majority of the patients were females (69%), and the mean age was 9.9 ± 4.7 years. 94% of the ANA tests were requested in pediatric rheumatology outpatient clinics, and 6% in general pediatrics and other outpatient clinics. Arthritis was the most common reason for ANA testing (41.7%). Of the patients who had ANA positivity, 37% were diagnosed with juvenile idiopathic arthritis (JIA), 15% with connective tissue diseases, 10% with autoinflammatory disease, and 7% with vasculitides. Positivity at 1/320 and 1/640 titers were more common in the patients diagnosed with autoimmune connective tissue diseases or JIA compared to the patients without these diagnoses (P =.009 and P =.013, respectively). The ANA test should be judiciously requested by pediatric rheumatologists, especially in suspected cases of autoimmune rheumatic disorders and JIA patients to aid in classification. Indiscriminate use of the ANA test for screening may potentially misguide clinicians. [ABSTRACT FROM AUTHOR]

Objectives: Inflammatory markers such as erythrocyte sedimentation rate (ESR) and C‐reactive protein (CRP) are poorly informative about interferon (IFN)‐related disorders. In these conditions, the measure of the interferon score (IS), obtained by measuring the expression of IFN‐stimulated genes, has been proposed. Flow cytometry‐based assays measuring sialic‐acid‐binding Ig‐like lectin 1 (Siglec‐1) expression could be a more practical tool for evaluating IFN‐inflammation. The study compared Siglec‐1 measures with IS and other inflammatory indexes. We compared Siglec‐1 measures with IS and other inflammatory indexes in real‐world paediatric rheumatology experience. Methods: We recruited patients with immuno‐rheumatological conditions, acute infectious illness and patients undergoing orthopaedic surgery as controls. Siglec‐1 expression was measured in all samples, and IS, ESR and CRP were also recorded if available. Results: Overall, 98 subjects were enrolled in the study, with a total of 104 measures of Siglec‐1. Compared with IS, Siglec‐1 expression showed good accuracy (86.0%), specificity (72.7%) and sensitivity (85.7%). The measure of the percentage of Siglec‐1‐positive cells performed best at low levels of IFN‐inflammation, while the measure of mean fluorescence intensity performed best at higher levels. Ex vivo studies on IFN‐stimulated monocytes confirmed this behaviour. There was no link between Siglec‐1 expression and either ESR or CRP, and positive Siglec‐1 results were found even when ESR and CRP were normal. A high Siglec‐1 expression was also recorded in subjects with acute infections. Conclusion: Siglec‐1 measurement by flow cytometry is an easy tool to detect IFN‐related inflammation, even in subjects with normal results of common inflammation indexes. [ABSTRACT FROM AUTHOR]

Background: Many children with rheumatic and musculoskeletal diseases are unrecognized. Identifying these children requires health care provider awareness, knowledge, and skills to recognize disease features and how (and when) to refer to specialist care. The aim of this paper is to highlight the need for better access to health care, review the essential role that education and virtual care play to address unmet need in low resource areas and especially to expand workforce capacity. Using collaborative partnerships, virtual platforms, and innovative assessment methods, musculoskeletal care and education can be delivered to reach a greater audience than ever before. Increased awareness through multiple initiatives and readily available resources are imperative to improve global rheumatology care. Conclusion: The needs of children with rheumatic diseases and musculoskeletal conditions are vastly underserved around the world resulting in preventable morbidity and mortality. Expanded implementation of virtual education and e-health care platforms provides an opportunity to increase access to care for children globally. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to examine the transition process of paediatric rheumatology patients from the Monash Children's Hospital (MCH) in Melbourne in order to identify areas that could be improved. Methods: Retrospective review of clinical data from the rheumatology database of paediatric rheumatology patients eligible for transition between January 2015 and September 2020. Results: One hundred and sixty‐five patients were included; 57 patients were transitioned. Of patients transitioned to an adult service, 38 (88%) were on medication and 14 (33%) had active disease. All patients transitioned to the general practitioner (GP) had inactive disease off medication. Juvenile idiopathic arthritis (JIA) (non‐systemic) was the most common diagnosis in patients transitioned. The mean age at which transition was first discussed was 18.0 years; the first referral was made at a mean of 18.3 years. The mean age at the first adult appointment was 18.5 years. Thirty‐nine (91%) patients had a referral completed and 8 (19%) had a transfer letter. Thirteen (93%) patients transferred to the GP had a transfer letter. Transfer documents to an adult public rheumatology service rated 4.3 for quality, compared to 5.5 to the GP. Transfer of care was confirmed in 40 (93%) patients transitioned to an adult service; however, correspondence was available for only 3 (7%). Conclusion: Although the transition process at MCH was adequate, it could be improved through earlier discussion of the process and improved referrals and documentation. A readiness‐to‐transfer checklist and a young adult clinic have the potential to improve the process of transition to adult rheumatology care. [ABSTRACT FROM AUTHOR]

Aim: This population-based study investigated the occurrence of capillary leak syndrome (CLS) in children with multisystem inflammatory syndrome in children (MIS-C), associated with COVID-19. We also examined associations between CLS and MIS-C disease severity. Methods: All eligible individuals aged 0-18 years, who were diagnosed with MIS-C in Skåne, southern Sweden, from 1 April 2020 to 31 July 2021, were studied. They were all included in the Pediatric Rheumatology Quality Register and clinical and laboratory data were compared between patients with and without CLS. Results: We included 31 patients (61% male) with MIS-C in the study. The median age at diagnosis was 10.6 years (range 1.99-17.15) and 45% developed CLS. All six patients who required intensive care had CLS. Patients with CLS also had a higher incidence of reduced cardiac function, measured as low ejection fraction. The CLS group exhibited significantly higher C-reactive protein values (p < 0.001) and N-terminal pro-Btype natriuretic peptide levels (p < 0.001), as well as lower platelet counts (p = 0.03), during the first week of treatment. Individuals with CLS also received more intense immunosuppression. Conclusion: CLS was a common complication of MIS-C in our study and these patients had a more severe disease course that required more intensive treatment. [ABSTRACT FROM AUTHOR]

Background: The clinical relevance of different antiphospholipid antibody (aPL) profiles, including low level anticardiolipin (aCL) and anti-β2-glycoprotein-I (aβ2GPI) antibodies, is ill-defined in the pediatric population. Our purpose is to describe the demographic, clinical, and laboratory characteristics of aPL positive pediatric patients based on different aPL profiles. Findings: In this single center retrospective cohort study, based on the screening of our pediatric (age ≤ 18) rheumatology electronic medical records (2016–2022), we identified patients who had at least one "positive" aPL (lupus anticoagulant [LA], aCL IgG/M, or aβ2GPI IgG/M) result. Patients were grouped into high- (LA positive and/or aCL/aβ2GPI IgG/M > 40U [ELISA]) and low-risk (LA negative and aCL/aβ2GPI IgG/M 20-39U) aPL profiles; those with persistently positive aPL were descriptively analyzed for demographic and clinical characteristics. Of 57 included patients, 34 (59%) had initial high- and 23 (40%) had initial low-risk profiles. Based on subsequent aPL results available in 42/57 (74%) patients, 25/27 (93%) in the high-, and 7/15 (47%) in the low-risk groups remained still positive. Of these 32 patients with persistently positive aPL, moderate-to-large vessel or microvascular thrombosis occurred in nine (28%) patients with high-risk and in none with low-risk aPL profiles; non-thrombotic aPL-related manifestations were reported in 15 (47%) patients with persistent aPL positivity. Conclusion: An initial high-risk aPL profile was persistent in approximately 90% of our cohort, a third of whom had thrombosis, and half had non-thrombotic aPL manifestations. Our results underscore the need for a large-scale effort to better characterize aPL-related manifestations in pediatric patients with persistent high-risk aPL-profiles. [ABSTRACT FROM AUTHOR]

Objective: Biologic therapy has changed the prognosis of patients with rheumatologic disease. Despite all benefits of the biological agents, adverse events may occur due to their long-term use. The aim of this study is to analyze the adverse events observed in pediatric patients who received biological treatment. Materials and Methods: This retrospective observational cohort study was conducted between January 2010 and January 2022. File records of 139 patients used biological agents for rheumatologic diseases in a pediatric rheumatology clinic were evaluated. Diagnosis, received treatment, the rationale for stopping treatment, requirement of tuberculosis prophylaxis, presence of an adverse event, and results were recorded. Results: The most used biological therapy was etanercept (41.7%). Anakinra, adalimumab, canakinumab were used in 30.9%, 27.3%, 23.7% of patients, and the others in less than 10%. Totally 491 adverse events (97.9/100 patient-years) were encountered during the duration of biological treatment. The most often adverse event was recurrent upper respiratory tract infection in the patients (31.9/100 patient-years). Elevated aminotransferase levels (10.4/100 patient-years), abdominal pain (7/100 patient-years), and headache (5.2/100 patient-years) were among the other common side effects. Isoniazid (INH) prophylaxis was needed before biological treatment in 20.9% of the patients. Tuberculosis developed in none of the patients followed-up for latent tuberculosis, however, it developed in a patient while receiving etanercept due to noncompliance with his scheduled outpatient visits during etanercept treatment. Conclusion: The most commonly used biological treatments were TNFi and IL-antagonists, and the majority of side effects were infections and laboratory abnormalities. Although the rate of serious adverse events is quite low, close follow-up of patients receiving biological therapy is very important. [ABSTRACT FROM AUTHOR]