Pediatric Rheumatology Centers Research Articles

P58 YOUNG PERSONS' PERSPECTIVES ON THE RESULTS OF A UK MULTI-CENTRE SURVEY ON VARIATIONS IN CLINICAL PRACTICE IN PAEDIATRIC RHEUMATOLOGY UNITS DURING THE COVID-19 PANDEMIC

Introduction/BackgroundA paediatric rheumatology multi-centre survey was conducted to understand the impact of COVID-19 on services and provide useful learning points that could change future practice. The survey focused on clinicians' views of the pandemic. The aim of this study was to gather the perspectives of young people regarding the results of the initial survey. This was done via a group discussion with members of the national youth advisory panel for adolescent rheumatology – Your Rheum. It is important when evaluating service provision that not only the clinician’s perspective but the patient’s voice is heard.Description/MethodThe initial survey was sent, in August 2021, to Consultants in all UK paediatric rheumatology centres, to the paediatric rheumatology trainees network and to the JIA topic specific group members via email. 20 staff responded (17 consultants and 3 trainees at registrar level) across the 4 UK nations. Results of the survey showed changes to the frequency of blood monitoring (n = 17), an increase in disease flares (n = 4) with all respondents reporting changes to the type of consultation used with a large increase in the use of virtual consultations. Increases in clinic waiting lists due to capacity issues were also reported (n = 7). 15 respondents reported changes in steroid usage for flare management with more ward based steroid injections performed and an increase in the use of oral steroids due to theatre unavailability.The online discussion group held with ‘Your Rheum’ members consisted of 9 Young People (YP) aged between 12-24 years. The discussion group was facilitated by a research assistant, paediatric rheumatology trainee and a consultant paediatric rheumatologist. A number of statements with Agree/Disagree/Unsure options were provided with an opportunity to vote on each statement. A follow up discussion was held after each statement around the issues raised. The online tool Mentimeter was used to form a word cloud on YP experiences of the pandemic which was followed by breakout rooms to facilitate further small group discussions.Discussion/ResultsIn contrast to the survey results which revealed that clinicians perceived that YP preferred virtual (telephone or video or combination of both) consultations, young people in ‘Your Rheum’ varied in their responses with the majority of comments describing that it depended on the clinical context (if unstable or stable disease) with virtual appointments being preferable when their disease was stable. YP also reported issues with virtual appointments including difficulties with phone signal, not being able to obtain a specific time for appointments (varied time slots), issues for YP with hearing difficulties as well as one YP commenting it is “easier to lie to the doctor and just say everything is going really well”. Negative points raised with face-face appointments included longer waiting times and the time spent travelling to appointments.The majority of young people agreed that changes to the normal pattern of blood monitoring was concerning as this could lead to a possible increase in flare of conditions. Specific comments including accessibility to appointments was difficult during the pandemic with the additional fear of getting COVID-19 if attending the hospital for blood monitoring.YP used a variety of words to explain their experiences of rheumatology services during the pandemic including: ‘rushed’, ‘frustrating’, ‘stressful’, ‘virtual’, ‘chaotic’, ‘poor communication’, ‘inaccessible’ but also ‘COVID safe’.Key learning points/ConclusionYoung people with rheumatic diseases offered different insights to the initial clinician survey findings highlighting the importance of considering their opinion as well as that of professionals when considering service provision and evaluation.Flexibility is essential for young people and a hybrid approach with virtual/face to face appointments is key. Overall YP prefer face-face but are willing to have virtual consultations depending on the nature of issues needing addressed and the stability of disease.Experiences of poor communication between YP and health professionals/hospital has always been a theme in their experiences to date but has worsened since COVID-19.YP within this group discussion preferred to have blood monitoring performed at regular frequencies (similar to pre-pandemic) but did have concerns about getting COVID-19 by attending these appointments during the pandemic. It is important that YP are encouraged and reassured about attending appointments whilst ensuring COVID-19 safe precautions.

OA32 Minimally invasive, ultrasound-guided tissue biopsies of synovial tissue in children with Juvenile Idiopathic Arthritis for research: a feasibility study

Introduction/BackgroundWhen investigating disease mechanisms, site-specific differences in immune cell phenotype and function have highlighted the need to analyse cellular and molecular mechanisms at the tissue site directly. In adults, the ability to obtain synovial tissue biopsies using ultrasound-guided techniques, combined with advanced tissue analytics, has revolutionised our understanding of the cellular ecosystem that operates within the joint and how it contributes to disease. However, a similar approach in paediatric disease is lacking. Description/MethodAims: 1) To describe the protocol for undertaking minimally invasive ultrasound-guided synovial tissue biopsies in children and young people with arthritis, for the purpose of research, alongside routine clinical care. 2) To investigate whether high-quality synovial tissue can be obtained that is suitable for downstream applications including single cell profiling technologies, histology and digital spatial profiling.Treatment-naïve children with a diagnosis of Juvenile Idiopathic Arthritis, who were being referred for a corticosteroid joint injection were recruited from two large UK Paediatric Rheumatology centres. We established a workflow pipeline for performing synovial tissue biopsies in child and young people with arthritis, using standardised procedures for biopsy and sample processing. Procedures were performed by experienced paediatric interventional radiologists with experience of joint biopsy for diagnostic purposes. Following a general anaesthetic, required as part of routine clinical care and the establishment of sterility, synovial fluid was aspirated. Needle-biopsies were undertaken from the same needle insertion site and subsequently corticosteroid was injected into the joint. Thickened synovium was graded via ultrasonography. Participating families completed questionnaires prior to and following synovial biopsy.Discussion/Results11 participants were recruited to the study over a nine month period, with a median age of 7 years (range 1-16 years); 91% were female. Samples obtained included core synovial biopsies, paired synovial fluid and peripheral blood. Synovial tissue fragments were processed for histology by formalin fixation and cryopreserved for downstream applications, including RNA sequencing and cell culture. Quality control indices included histological analysis to ensure the biopsied material was characteristically synovium and to grade the severity of inflammation. No significant complications were reported; however, one child had a mild haemarthrosis controlled with cold saline wash out and cold compresses.Key learning points/ConclusionObtaining biopsies of synovial tissue in children with Juvenile Idiopathic Arthritis for the purpose of research, alongside clinical care is feasible. Analysis of tissue direct from the site of inflammation with single-cell RNA sequencing in children is achievable.

Juvenile Fibromyalgia in Patients With Juvenile Idiopathic Arthritis: Utility of the Pain and Symptom Assessment Tool.

To evaluate the proportion of children with juvenile idiopathic arthritis (JIA) who met criteria for comorbid juvenile fibromyalgia (FM) using the Pain and Symptom Assessment Tool (PSAT), and to identify clinical and demographic differences among JIA patients with and without juvenile FM. Patients ages 11-17 years with JIA were recruited from 4 North American pediatric rheumatology centers. Each patient completed the PSAT. Additional clinical and disease activity measures included pain visual analog scale, patient global assessment of disease activity (PtGA) and physician global assessment of disease activity (PhGA), the Functional Disability Inventory (FDI), and the Pain Catastrophizing Scale in children. Of 129 patients, 11 met criteria for juvenile FM. FDI scores were markedly higher in patients who tested positive for juvenile FM, with a mean of 24.8 compared to 6.9 in patients without juvenile FM (P < 0.001). Pain catastrophizing scores were also significantly higher, by ~14 points, in patients with juvenile FM. There was a significant tendency for patients to give higher disease activity scores than physicians, which was more marked among patients with juvenile FM. In patients with juvenile FM, PtGA scores exceeded PhGA scores by a mean of 3.7, compared to a mean of 0.7 among patients without juvenile FM (P < 0.001). A minority of JIA patients (8.5%) met criteria for juvenile FM. This group demonstrated markedly more functional impairment. PtGA scores were strikingly higher than PhGA scores among patients with JIA who met juvenile FM criteria, suggesting that providers might consider a more expansive approach to chronic pain and non-musculoskeletal symptom assessment and treatment in JIA patients.

Real-life vaccination coverage in Slovak children with rheumatic diseases.

BackgroundEvidence-based recommendations for vaccination of patients with pediatric rheumatic diseases (PRDs) are available, their implementation in practice is unknown.ObjectivesTo analyze real-life vaccination coverage in children with PRDs and identify reasons for incomplete vaccination.MethodsUp-to-date information on vaccination status of Slovak children followed at a tertiary pediatric rheumatology center was retrieved from pediatricians over an 18-month period and compared to the standard Slovak Immunization Schedule. Reasons for missed vaccinations were analyzed retrospectively.ResultsVaccination records of 156 patients (median age 10 years, 2–18) with PRDs (JIA n = 108, systemic diseases n = 21, autoinflammatory diseases n = 16, uveitis n = 9, others n = 2) were available for analysis. 117 (75.0%) were completely vaccinated, 2 (1.3%) had not received any vaccine due to reasons unrelated to PRD. 37 (23.7%) remaining patients missed altogether 48 mandatory vaccinations. In 58.3% (n = 28, in 24 patients) no PRD related reasons for missing vaccinations were identified. Only 20 vaccinations (18 live-attenuated and 2 non-live in 19 patients) were missed due to ongoing immunosuppressive treatment or PRD activity. Patients aged 11–14 years were more likely to be incompletely vaccinated than other age groups (48.8% vs. 15.9%, p < 0.001), mainly due to missed MMR booster. Systemic immunosuppressive treatment was a significant predictor for incomplete vaccination status (OR 5.03, 95% CI 1.13–22.31, p = 0.03).ConclusionFull vaccination is possible in a high proportion of PRD patients. In addition to immunosuppressive therapy, reasons unrelated to PRDs are a frequent and possibly inadequate cause of missed vaccinations. Periodic vaccination status assessments are needed in pediatric rheumatology care.

What are the factors associated with the duration of remission of intra-articular corticosteroid injection in juvenile idiopathic arthritis?

Intra-articular corticosteroid injection (IACI) is generally used in the management of juvenile idiopathic arthritis (JIA) to obtain rapid relief of active synovitis and functional recovery and to prevent the need for regular systemic therapy. The aim of this study was to investigate the outcome of IACI treatment and the factors associated with remission of synovitis. The clinical records of JIA patients who received IACI between January 2014 and December 2020 in two pediatric rheumatology centers were reviewed. The procedure was evaluated in terms of efficacy, factors that may affect the duration of remission, procedural and drug-related complications. During the study period, 134 patients received 227 injections and 37 joints were injected more than once. One hundred and six (79%) patients had persistent oligoarticular disease. At the time of injection, all patients were receiving non-steroidal anti-inflammatory drugs, 74 patients were on methotrexate, and 14 patients were on biologics. The median duration of remission without exacerbation of synovitis treated with IACI was 15 (range 1-64) months. The inactivity rate was 81% at the 6th month after the injection. It has been shown that being less than 7years old at disease onset and low initial CRP levels were correlated with a long remission period (p < 0.05). Despite the differences were not statistically significant, the duration of remission was longer in boys, in ANA positives, in HLA-B27 negatives, in patients with concurrent methotrexate treatment and in patients not receiving biologic therapy (p > 0.05). Only two patients (1.5%, 95% CI - 0.6 to 3.5) developed cutaneous hypopigmentation and subcutaneous atrophy as side effects of injection. Intra-articular corticosteroid injection was more effective especially in patients with low initial CRP levels and younger than 7years of age. The duration of remission was longer in these patients. Key Points • Intra-articular corticosteroid injection is an effective method for controlling joint inflammation and achieving long-term remission without significant side effects. • Intra-articular corticosteroid injection can be preferred in all forms of juvenile idiopathic arthritis as primary therapy or to relieve the patient while waiting for the effect of systemic agents or to avoid increasing the dose of systemic drugs. • It can be recommended as a treatment option at any stage of the disease, especially in young patients and patients with low initial CRP values.

Case Series of Myocarditis Following mRNA COVID Vaccine Compared to Pediatric Multisystem Inflammatory Syndrome: Multicenter Retrospective Study.

Introduction: Since the development of COVID-19 vaccines, more than 4.8 billion people have been immunized worldwide. Soon after vaccinations were initiated, reports on cases of myocarditis following the second vaccine dose emerged. This study aimed to report our experience with adolescent and young adults who developed post-COVID-19 vaccine myocarditis and to compare these patients to a cohort of patients who acquired pediatric inflammatory multisystem syndrome (PIMS/PIMS-TS) post-COVID-19 infection. Methods: We collected reported cases of patients who developed myocarditis following COVID-19 vaccination (Pfizer mRNA BNT162b2) from all pediatric rheumatology centers in Israel and compared them to a cohort of patients with PIMS. Results: Nine patients with post-vaccination myocarditis were identified and compared to 78 patients diagnosed with PIMS. All patients with post-vaccination myocarditis were males who developed symptoms following their second dose of the vaccine. Patients with post-vaccination myocarditis had a shorter duration of stay in the hospital (mean 4.4 ± 1.9 vs. 8.7 ± 4.7 days) and less myocardial dysfunction (11.1% vs. 61.5%), and all had excellent outcomes as compared to the chronic changes among 9.2% of the patients with PIMS. Conclusion: The clinical course of vaccine-associated myocarditis appears favorable, with resolution of the symptoms in all the patients in our cohort.

Baseline characteristics of children with juvenile dermatomyositis enrolled in the first year of the new Childhood Arthritis and Rheumatology Research Alliance registry

BackgroundTo report baseline characteristics, patient reported outcomes and treatment of children with Juvenile Dermatomyositis (JDM) in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.MethodsChildren 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.ResultsIn 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.ConclusionsIn 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.

Patterns and rates of confirmed transfer of care of patients with juvenile idiopathic arthritis at a tertiary paediatric rheumatology centre.

Disease activity in juvenile idiopathic arthritis (JIA) commonly persists into adulthood. Transfer of JIA patients to adult healthcare services can be challenging, with prior studies showing poor rates of success. This audit sought to examine characteristics of patients undergoing transfer of care within the rheumatology unit at the Royal Children's Hospital in Melbourne, with the aim of identifying areas for improvement. Specifically, we sought to determine the rate at which confirmation of established care with an adult service (confirmed transfer of care) was documented in the patient chart. Patients with a diagnosis of JIA who turned 18 years of age between 2012 and 2019 were identified. A chart review was undertaken to collect relevant data. One hundred and seventy-seven patients were identified. In all, 64% (114/177) were referred for adult care. The commonest JIA subtypes referred were seronegative polyarticular (35/114; 30.7%) and oligoarticular JIA (22/114; 19.3%). Documentation of confirmed transfer of care occurred in 62.3% (71/114), with correspondence received from adult services in 49.1% (56/114). There was no difference in rate of return correspondence from public versus private providers (45% vs 53.8%; P = 0.38). The use of 'backstop appointments' was more likely in those with confirmed transfer of care (66% vs 30%; P = 0.0002). Lack of confirmed transfer of care for JIA patients is common and carries a risk of suboptimal outcomes. Strategies to improve communication with adult services, the routine use of 'backstop' appointments and vigilance regarding potential loss to follow up at the time of transfer would minimise this risk.

OP0056 “BECAUSE THAT’S THEIR JOB, ISN’T IT?”: EXPLORING CHILDREN AND YOUNG PEOPLES’ EXPERIENCES OF AND PERSPECTIVES ABOUT PAIN COMMUNICATION IN PAEDIATRIC RHEUMATOLOGY IN THE UK

BackgroundPain communication should be an integral part of every clinical consultation, particularly in paediatric rheumatology where children/young people often present complex long-term conditions in which chronic pain is a feature. Researchers investigating pain communication in paediatric healthcare encounters have been focused on healthcare professionals, yielding inconsistent findings about the occurrence and nature of pain discussions with children/young people. There has been limited research examining children/young peoples’ own experiences and perspectives on this in the literature to date.ObjectivesThe objective of this study was to investigate children/young peoples’ experiences and perceptions of communicating about pain with paediatric rheumatology healthcare professionals in the UK.MethodsTwenty-six children/young people were recruited from three UK paediatric rheumatology centres. Data were collected using semi-structured telephone interviews between April-October, 2021. A framework analysis approach was used to explore similarities and divergences in participants’ narrative accounts.ResultsThe mean age of children/young people was 14.0 years (SD=3.6 years, Range= 6-18 years, 58% female). Diagnoses included; Juvenile Idiopathic Arthritis, Chronic Regional Pain Syndrome, diffuse idiopathic chronic pain, localised idiopathic pain, hypermobility (including Ehlers Danlos Syndrome) and Raynaud’s disease.Findings are organised into four themes.1) Nature and focus of appointments. Children/young people talked about pain with several professionals from the team. Participants reported that pain conversations predominantly occurred during physical examinations.2) Co-ordination of pain communication. Children/young people identified how professionals mostly started pain conversations. They explained how they were often asked to verbally rate pain rather than use a written assessment tool. For some, questions about pain were directed to parents. Participants reported that this was problematic as parents “can’t feel” pain.3) Reflections on pain communication. There were expectations that pain should always be asked about as it was considered a main reason for a consultation. Participants discussed how these conversations gave them an opportunity to “get it off their chest” and made them feel “reassured” that professionals “care”. Being asked about pain reminded them that they were different to peers and they were concerned it could highlight “something else is wrong”. Children/young people talked about how it became easier to talk about pain with familiar professionals.4) Moving forward after pain communication. Children/young people discussed how professionals could give mixed messages about how to manage pain at home following consultations, offering advice which was difficult to put into practice (e.g. “doing too much” vs “not doing enough”). Children/young people expressed their need to discuss the emotional as well as the physical effects of their pain.ConclusionThese study findings highlight a range of effective and ineffective pain communication approaches from the experiences and perspectives of children/young people. These will be used to create recommendations for improving the communication of chronic pain in paediatric rheumatology in the future, in a way that is acceptable and valuable to children/young people.AcknowledgementsThe authors would like to thank the children/young people for kindly taking the time to share their experiences and perceptions about their interactions with healthcare professionals in paediatric rheumatology. The views expressed herein are those of the authors and not necessarily those of the National Health Service, the National Institute for Health Research, or the UK Department of Health. This work was supported by a Foundation Fellowship award from Versus Arthritis (Grant 22433). Aspects of this work were also supported by funding from the Centre for Epidemiology Versus Arthritis (Grant 20380) and the NIHR Manchester Biomedical Research Centre.Disclosure of InterestsNone declared.

The Effects of a Web-Based Tool for Parents of Children With Juvenile Idiopathic Arthritis: Randomized Controlled Trial.

BackgroundJuvenile idiopathic arthritis (JIA) is a group of autoinflammatory diseases that cause pain and disability if not controlled by treatment. Parenting a child with JIA is stressful for parents, who express concerns about their child’s treatment and may experience anxiety and powerlessness concerning their child’s illness. Parenting stress is greater in parents of children with chronic illness than in those with healthy children and is related to poorer psychological adjustment in both parents and children. It is therefore important to develop interventions to support parents. This paper reports the evaluation of a web-based tool that provides information and practical skills to help increase parents’ confidence in managing their child’s illness and reduce parenting stress.ObjectiveThe aim of this study is to evaluate the benefits of a web-based tool (WebParC) for parents of children with recently diagnosed JIA.MethodsA multicentered randomized controlled trial was conducted at pediatric rheumatology centers in England. We recruited parents of children aged ≤12 years who had been diagnosed with JIA within the previous 6 months. They were randomized to the intervention (WebParC access plus standard care) or the control (standard care alone) and followed up 4 months and 12 months after randomization. Where both parents participated, they were randomized by household to the same trial arm. The WebParC intervention consists of information about JIA and its treatment plus a toolkit, based on cognitive behavioral therapy, to help parents develop skills to manage JIA-related issues. The primary outcome was the self-report Pediatric Inventory for Parents measure of illness-related parenting stress. The secondary outcomes were parental mood, self-efficacy, coping, effectiveness of participation in their child’s health care, satisfaction with health care, and child’s health-related quality of life.ResultsA total of 203 households comprising 220 parents were randomized to the intervention (100/203, 49.3%) or control (103/203, 50.7%) arm. Follow-up assessments were completed by 65.5% (133/203) of the households at 4 months (intervention 60/100, 60%, and control 73/103, 70.9%) and 61.1% (124/203) of the households at 12 months (intervention 58/100, 58%, and control 66/103, 64.1%). A main effect of the trial arm was found on the Pediatric Inventory for Parents: the intervention participants reported less frequency (subscales communication F1,120627=5.37; P=.02, and role function F1,27203=5.40; P=.02) and difficulty (subscales communication F1,2237=7.43; P=.006, medical care F1,2907=4.04; P=.04, and role function F1,821=4.37, P=.04) regarding illness-related stressful events than the control participants.ConclusionsThe WebParC website for parents of children with JIA reduced illness-related parenting stress. This web-based intervention offers a feasible preventive approach for parents of children with JIA and potentially could be adapted and evaluated for parents of children with other chronic illnesses.Trial RegistrationInternational Standard Randomized Controlled Trial Number (ISRCTN) 13159730; http://www.isrctn.com/ISRCTN13159730

P017 Variations in clinical practice in paediatric rheumatology units as a result of the COVID-19 pandemic: Initial findings of a UK wide Multi-Centre Survey

Background/AimsTo understand the impact of COVID-19 on UK paediatric rheumatology services, to determine the learning points that could change future practice and provide information for trainees to plan further quality improvement projects.MethodsSurvey details with a Microsoft forms link were emailed to consultants in each UK paediatric rheumatology centre. Consultants were asked to forward the survey onto any regional units linked. The survey was also sent to the paediatric rheumatology trainee’s network. The questionnaires covered blood monitoring, joint injections, oral corticosteroid use, modes of consultations, positive and negative impacts and what changes should be carried forward.Results20 staff responded to the survey; 17 consultants and three trainees (registrar level). 85% (17) reported changes had occurred to the frequency of blood monitoring to rheumatology patients on Disease Modifying Anti-Rheumatic Drugs (DMARDs) and/or biologics during the pandemic. 60% (12) reported this was due to patient/family reluctance to attend or due to unavailability of appointments. 75% (15) recorded no complications following changes of blood frequency on disease activity or flare. Of those that did have a flare, a number of factors were described including: parents not contacting their service, patient/family discontinuation of treatment, lack of clinic attendance, running out of treatment and safety concerns re prescribing from primary care or local department. One patient on methotrexate experienced significantly deranged liver function tests.75% (15) reported changes with provision of joint injections due to theatre unavailability. As a consequence, 60% (12) reported an increase in the use of oral corticosteroids with 20% (4) performing more ward-based injections with local anaesthetic or Entonox on younger children. 100% (20) changed their mode of consultation. Between 5 and 40% of all appointments are now virtual (telephone or video) and 60-95% are face-face. 60% (12) report that frequency of review appointments has now returned to normal. Major disruption has occurred in seeing new patients due to a backlog of patients created by the pandemic. Some patients have been referred to units with possible arthritis that haven’t been examined or seen face-face in primary care prior to referral.ConclusionCOVID-19 caused significant changes to UK paediatric rheumatology services. The results highlighted the use of virtual consultations where appropriate, consideration of joint injections without general anaesthetic as much as possible, enabling virtual teaching/conferences and also raising the need to review blood monitoring frequency for patients on DMARDs/biologics. Longer waiting lists, increased anxiety amongst children and young people, increase in disease activity and the impact on learning opportunities for trainees were concerning negative aspects of the pandemic. It is likely the impact of these negative consequences will continue to be experienced for some time. Future planning and consideration is required to minimise the negative impact of these aspects on our patients and colleagues.Disclosure D. McLaughlin: None. S. Deepak: None.

P171 Reasons for stopping biologic and non-biologic disease-modifying treatments in children and young people managed by a tertiary paediatric rheumatology centre

Abstract Background/Aims The Paediatric Rheumatology Department at Newcastle upon Tyne Hospitals looks after children and young people (CYP) across the North East and Cumbria region. The centre treats around 220 patients (250 active prescriptions) using homecare services to deliver injectable medicines. These prescriptions are for five medicines: methotrexate, adalimumab, anakinra, tocilizumab and etanercept. This project aimed to analyse the reasons treatments were stopped and identify potentially-modifiable factors to optimise treatment. Methods Patients were identified from the departmental database. Cases were included if they stopped one of the 5 subcutaneous treatments during the period 1st July 2020 to 30th June 2021. Demographic details, the underlying diagnoses and reasons for stopping treatment were extracted from electronic patient records. Results During the 12-month period analysed, 109 prescriptions were stopped. This represented 104 unique patients (56% female). The commonest indications for treatment were juvenile idiopathic arthritis in 80% (87/109), idiopathic uveitis in 11% (12/109) and autoinflammatory diseases in 5.5% (6/109). The following treatments were stopped: methotrexate in 47 patients, adalimumab in 43, etanercept in 7, tocilizumab in 6 and anakinra in 6. The reasons for stopping prescriptions are shown in Table 1. After excluding 17 cases whose prescriptions were taken over by another unit, 92 prescriptions were stopped for clinical reasons. Of these, 28% were stopped due to side effects. All 19 patients who suffered nausea or vomiting, and all 5 patients with abnormal liver function tests, were receiving methotrexate. The 2 patients who stopped treatment due to neutropenia were both receiving tocilizumab. Almost 23% (21/92) of prescriptions were stopped due to inefficacy. All cases were switched to another biologic drug. Conclusion A substantial proportion of subcutaneous disease-modifying treatments were stopped due to inefficacy or side effects. Stopping and switching treatments represents a period of uncertainty for CYP and their families, with potential impact on disease control and quality of life. Management could be improved by a stratified medicine approach, using biomarkers to predict patients at highest risk of side effects or most likely to respond to a particular treatment. This is a key aim of current research. Disclosure K. Hartley: None. E.S. Sen: None.

The Multifaceted Presentation of the Multisystem Inflammatory Syndrome in Children: Data from a Cluster Analysis.

Background: The aim of this study was to evaluate the outcomes of patients with the multisystem inflammatory syndrome in children (MIS-C) according to phenotypes of disease and define the prognostic factors for the severe course. Methods: This cross-sectional study included 293 patients with MIS-C from seven pediatric rheumatology centers. A two-step cluster analysis was performed to define the spectrum of disease and their outcomes were compared between each group. Results: Four subgroups were identified as follows: cluster I, predominantly Kawasaki-like features (n = 100); cluster II, predominantly MAS-like features (n = 34); cluster III, predominantly LV dysfunction (n = 47); cluster IV, other presentations (n = 112). The duration of fever was longer in cluster II and the length of hospitalization was longer in both clusters II and III. Laboratory findings revealed lower lymphocyte and platelet counts and higher acute phase reactants (APRs) in cluster II, while patients in cluster IV showed less inflammation with lower APRs. The resolution of abnormal laboratory findings was longer in clusters II and III, while it was shortest in cluster IV. Seven patients died. Among them, four belonged to cluster II, while three were labeled as cluster III. Patients with severe course had higher levels of neutrophil–lymphocyte ratio, mean platelet volume, procalcitonin, ferritin, interleukin-6, fibrinogen, D-Dimer, BNP, and troponin-I, and lower levels of lymphocyte and platelet counts. Conclusion: As shown, MIS-C is not a single disease presenting with various clinical features and outcomes. Understanding the disease spectrum will provide individualized management.

Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches.

Background: Systemic Juvenile Idiopathic Arthritis (SJIA)/Pediatric Still’s disease is associated with different phenotypes and outcomes from currently available treatments. Methods: A review of opinion, based on personal experience in a reference pediatric rheumatology center and key publications, to explore the most important questions regarding disease heterogeneity and treatment approaches. Results: A few situations deserve particular attention: 1/patients with recent-onset SJIA who may benefit from a treat-to-target approach with a key place for interleukin (IL)-1 inhibition; 2/SJIA patients refractory to Il-1 and IL-6 antagonists in whom several options may be discussed, including thalidomide or allogeneic hematopoietic stem cell transplantation; 3/SJIA patients with macrophage activation syndrome who may benefit from both well-used classical treatment and innovative approaches, such as anti-interferon gamma therapy or Janus Kinase (JAK) inhibitors; 4/SJIA with severe lung involvement, 5/SJIA patients who achieve complete remission on treatment, with some recent evidence that treatment may be reduced in intensity but not so easily withdrawn. Conclusions: a case-by-case discussion with expert teams is recommended in this heterogeneous, often difficult-to-treat population of patients.

Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS): a Brazilian cohort

BackgroundPaediatric inflammatory multisystem syndrome (PIMS) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been described since mid-April 2020 with the first reports coming from Europe. Our objective was to describe the characteristics of patients among the Brazilian population.MethodsA multicenter retrospective study was conducted with the participation of five pediatric rheumatology centers in Brazil during the period from March to November 2020. Children and adolescents with PIMS temporally associated with SARS-CoV-2 (TS) who met the definition criteria for the disease according to the Royal College of Paediatrics and Child Health were included. Demographic, clinical, laboratory, therapeutic characteristics and molecular and serological diagnosis of SARS-CoV-2 infection were described.ResultsFifty-seven children and adolescents with PIMS-TS were evaluated, 54% female, with a median age of 8 (3–11) years. Most (86%) were previously healthy, with asthma being the main comorbidity, present in 10% of the patients. Fever was the main manifestation, present in all patients, followed by mucocutaneous and gastrointestinal features, present in 89% and 81% of the patients, respectively. Myocarditis occurred in 21% of the patients and in 68% of them required intensive care. The Kawasaki disease phenotype occurred in most patients (77%). All patients had elevated inflammatory markers, with elevated CRP being the most found (98%). Anemia and lymphopenia were present in 79% and 72%, respectively. Laboratory evidence of SARS-CoV-2 was found in 77% of the patients, with 39% positive RT-PCR and 84% positive serology for SARS-CoV-2. An immunomodulatory treatment was performed in 91% of the patients, with 67% receiving intravenous immunoglobulin (IVIG) associated with glucocorticoid, 21% receiving IVIG, and 3.5% receiving glucocorticoid. The median length of hospitalization was 10 days.ConclusionsThis study showed a high morbidity of PIMS-TS in Brazilian children, with a prolonged length of hospitalization and a high rate of admission to pediatric intensive care unit. Multicenter prospective studies are needed to assess the morbidity of the disease in the medium and long term.

The efficacy and safety of intra-articular injection of triamcinolone acetonide versus triamcinolone hexacetonide for treatment of juvenile idiopathic arthritis

ObjectivesJuvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease. Intra-articular corticosteroids joint injection (IAJI), with triamcinolone hexacetonide (TH) or triamcinolone acetonide (TA), is an effective additional treatment for oligo and polyarticular JIA. Previous studies have shown the benefits of TH over TA; however, TA is still used in many pediatric rheumatology centers. Our unit has experience with both regimens, and therefore we aimed to compare the efficacy and safety of TA versus TH for JIA patients.MethodsChart review of JIA patients who were randomly (based on drug availability) treated with TA or TH IAJI during 2010–2019. Primary outcomes for efficacy were defined as full recovery from arthritis one month after IAJI and a relapse rate of arthritis 3 months after IAJI. Primary outcome for safety was defined as the occurrence of adverse events (AEs) during the follow up period after IAJI.ResultsOverall, 292 joints of 102 JIA patients were treated (138 TA/154 TH joints). Complete recovery after one month was documented in 107 (69.6%) of TA treated joints and 96 (69.5%) of TH treated joints (P = 0.232). However, rate of relapse after 3 months was significantly higher for TA treated joints (27 (20.1%) vs. 13 (8.8%), respectively, P < 0.01). No AEs were documented except minor scars at four joint injection sites.ConclusionThe recovery from arthritis was similar (~ 70%) with both regimens, however relapse rate was more than double in TA as compared to TH injected joints. These findings are important due to a contemporary shortage of TH in the US market.

Current practice of transitional care for adolescents and young adults in Swiss paediatric and adult rheumatology centres.

About half of all children with rheumatic diseases need continuous medical care during adolescence and adulthood. A good transition into adult rheumatology is essential. Guidelines for a structured transition process have therefore been recommended by the European League Against Rheumatism (EULAR) and the Paediatric Rheumatology European Society (PReS). However, implementation of these guidelines requires resources often not available in a busy clinical practice. To assess the current practice of transitional care in Switzerland in relation to EULAR/PReS recommendations and to describe gaps and challenges in following the recommendations. All paediatric Swiss rheumatology centres and their collaborating adult centres offering a transition service to adult care were invited to participate in this survey. The responsible paediatric and adult rheumatologist of each centre was interviewed separately using a structured manual addressing the EULAR/PReS transitional care recommendations. All 10 paediatric and 9 out of 10 adult rheumatologists agreed to participate. Centres varied in the number of patients in transition, from n = 0 to n = 111. The following EULAR/PReS recommendations were implemented and applied in most centres: continuity in the healthcare team, consultations focused on adolescents and young adults, joint consultations between the paediatric and adult rheumatologist, and access to the EULAR website. Only rarely did a centre have a written transition policy or evaluate their transitional care programme. The vast majority of the interviewees had no specific training in adolescent health. Most centres rated their transitional care performance as very good. Transition in Switzerland is not uniform and consequently the implementation of the EULAR/PReS recommendations is variable in Swiss rheumatology centres. Skills of healthcare professionals, continuity between clinical settings, size of the centres, and hospital focus on the needs of adolescents and young adults may represent key predictors of successful transitional care for patients with chronic rheumatic diseases. Future studies should examine these variables.

Low disease activity state in juvenile-onset systemic lupus erythematosus.

To determine the rate of achieving The Lupus Low Disease Activity State (LLDAS) in children with systemic lupus erythematosus (SLE) for tracing pertinent treatment modalities. A total of 122 juvenile-onset SLE (jSLE) patients from six pediatric rheumatology centers in Turkey were enrolled in the study. LLDAS-50 was defined as encountering LLDAS for at least 50% of the observation time. According to the achievement of LLDAS-50, clinical features, immunological profiles, and treatments of patients with jSLE have been revealed. LLDAS of any duration was achieved by 82% of the cohort. Although only 10.8% of the patients achieved remission, 68.9% reached LLDAS-50. A significant difference was found between patients who reached LLDAS-50 and those who did not, in terms of the time to reach low-dose corticosteroid treatment (p = 0.002), the presence of subacute cutaneous findings (p = 0.007), and the presence of proteinuria (p = 0.002). Both of the groups were under similar treatment approaches. However, the number of patients being treated with corticosteroids at the last visit was found to be significantly higher in patients who achieved LLDAS-50 (p<0.001). Targeting LLDAS in jSLE, even with long-term, low-dose corticosteroid use, seems to be an achievable goal in clinical practice.

A Population-based Study of Grade 12 Academic Performance in Adolescents With Childhood-onset Chronic Rheumatic Diseases.

The aims of this study were (1) to compare grade 12 standardized test results of patients diagnosed with childhood-onset chronic rheumatic diseases (ChildCRD) and unaffected peers; and (2) to identify factors associated with test results of patients with ChildCRD and unaffected peers. This was a population-based retrospective cohort study. All patients with ChildCRD (juvenile arthritis and systemic autoimmune rheumatic diseases) from the only pediatric rheumatology center in Manitoba for birth cohorts January 1979 to December 1998 were linked to the provincial administrative databases containing records of healthcare use and education outcomes. Patients were matched by age, sex, and postal codes to their peers who did not have ChildCRD. The primary outcomes were the grade 12 Language Arts Achievement Index (LAI) and the Math Achievement Index (MAI) scores. ChildCRD, sociodemographic, and mental health factors were tested for their associations with LAI and MAI scores using multivariable linear regression. Five hundred and forty-one patients with ChildCRD were matched to 2713 unaffected peers. Patients with ChildCRD had lower LAI and MAI scores compared to their peers. More patients with ChildCRD failed or did not take the language arts (51% vs 41%, P < 0.001) and math (61% vs 55%, P = 0.02) tests. On multivariable analysis, ChildCRD, lower socioeconomic status, younger maternal age at first childbirth, family income assistance, involvement with child welfare services, and mental health morbidities (between ChildCRD diagnosis and standardized testing), were associated with worse LAI and MAI results. This population-based study showed that patients with ChildCRD performed less well than their peers on grade 12 standardized testing, independent of sociodemographic and mental health comorbidities.

The clinical course and short-term health outcomes of multisystem inflammatory syndrome in children in the single pediatric rheumatology center

ABSTRACT Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition resulting in excessive response of the immune system after SARS-CoV-2 infection. We report a single-center cohort of children with MIS-C, describing the spectrum of presentation, therapies, clinical course, and short-term outcomes. Methods This is a prospective observational study from to a tertiary pediatric rheumatology center including patients (aged 1 month to 21 years) diagnosed with MIS-C between April 2020-April 2021. Demographic, clinical, laboratory results and follow-up data were collected through the electronic patient record system and analyzed. Results A total of 67 patients with MIS-C were included in the study. Fever was detected in all patients; gastrointestinal system symptoms were found in 67.2% of the patients, rash in 38.8%, conjunctivitis in 31.3%, hypotension in 26.9% myocarditis, and/or pericarditis in 22.4%, respectively. Respiratory symptoms were only in five patients (7.5%). Kawasaki Disease like presentation was found 37.3% of the patients. The mean duration of hospitalization was 11.8 7.07 days. Fifty-seven patients (85%) received intravenous immunoglobulin (IVIG), 45 (67%) received corticosteroids, 17 (25.3%) received anakinra, and one (1.5%) received tocilizumab. Seven of the patients (10.4%) underwent therapeutic plasma exchange (TPE). In 21 (31.3%) patients, a pediatric intensive care unit (PICU) was required in a median of 2 days. The first finding to improve was fever, while the first parameter to decrease was ferritin (median 6.5 days (IQR, 4–11.2 days)). Sixty-five patients were discharged home with a median duration of hospital stay of 10 days (IQR, 7–15 days). Conclusion Patients with MIS-C may have severe cardiac findings and intensive care requirements in admission and hospital follow-up. The vast majority of these findings improve with effective treatment without any sequelae until discharge and in a short time in follow-up. Although the pathogenesis and treatment plan of the disease are partially elucidated, follow-up studies are needed in terms of long-term prognosis and relapse probabilities.