Clinical Characteristics of Adolescents With Juvenile Idiopathic Arthritis Transitioning to Adult Rheumatology Care in Canada: Results From the CAPRI Registry.

OBJECTIVE: Using Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) juvenile idiopathic arthritis (JIA) registry data, we describe (1) clinical characteristics of patients with JIA transitioning to adult care, (2) prevalence of disease-related damage and complications, and (3) changes in disease activity during the final year prior to transfer. METHODS: Registry participants who turned 17 years between February 2017 and November 2021 were included. Clinical characteristics and patient-reported outcomes (PROs) at the last recorded pediatric rheumatology visit, and changes observed in the year prior to that visit were analyzed. Physicians completed an additional questionnaire characterizing cumulative disease-related damage and adverse events by age 17 years. RESULTS: At their last visit, 88 of 131 participants (67%) had inactive and 42 (32%) had active disease. Overall, 96 (73%) were on medications and 41 (31%) were on biologic disease-modifying antirheumatic drugs. Among 80 participants for whom the additional questionnaire was completed, 26% had clinically detected joint damage, 31% had joint damage on imaging, 14% had uveitis, and 7.5% had experienced at least 1 serious adverse event. During the final year, 44.2% of patients were in remission, 28.4% attained inactive disease, and 27.4% became or remained active. Mean scores of PROs were stable overall during that last year, but a minority reported marked worsening. CONCLUSION: A substantial proportion of youth with JIA transitioning to adult care in Canada had a high disease burden, which was reflected by their degree of disease activity, joint damage, or ongoing medication use. These results will inform pediatric and adult providers of anticipated needs during transition of care.

Comparing Canadian paediatric rheumatology practice to the 2019 ACR Juvenile Idiopathic Arthritis guidelines: results from the CAPRI Registry.

OBJECTIVE: To identify differences between baseline Canadian JIA practices and the 2019 ACR guidelines for JIA. METHODS: Canadian paediatric rheumatologists were surveyed for their opinions on reasonable a priori target adherence rates for JIA guideline recommendations. Prospectively collected data for 266 newly diagnosed children from 2017 to 2019 were analysed to calculate observed adherence rates. Kaplan-Meier survival curves were used to estimate the cumulative incidence of starting synthetic or biologic DMARDs (sDMARD or bDMARD, respectively) for different patient groups. RESULTS: A total of 25/61 (41%) eligible physicians answered the survey. Most survey respondents (64%) felt that adherence targets should vary depending on the strength of the recommendation and quality of evidence, from a mean of 84% for strong recommendations with high-quality evidence to 29% for conditional recommendations with very low-quality evidence. Data showed 13/19 (68%) recommendations would have met proposed targets and 10/19 (53%) had ≥80% observed adherence. Exceptions were the use of subcutaneous vs oral MTX (53%) and infrequent treatment escalation from NSAIDs to bDMARDs in patients with sacroiliitis (31%) or enthesitis (0%). By 12 weeks, 95% of patients with polyarthritis received sDMARDs, 38% of patients with systemic JIA received bDMARDs and 22% of patients with sacroiliitis received bDMARDs. CONCLUSION: Canadian paediatric rheumatology practices were in line with many 2019 JIA guideline recommendations before their publication, except for frequent use of oral MTX and infrequent direct escalation from NSAIDs to bDMARDs in sacroiliitis and enthesitis.

A decade of progress in juvenile idiopathic athritis treatments and outcomes in Canada: results from ReACCh-Out and the CAPRI registry.

OBJECTIVE: To assess changes in juvenile idiopathic arthritis (JIA) treatments and outcomes in Canada, comparing a 2005-2010 and a 2017-2021 inception cohorts. METHODS: Patients enrolled within three months of diagnosis in the Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh-Out) and the Canadian Alliance of Pediatric Rheumatology Investigators Registry (CAPRI) cohorts were included. Cumulative incidences of drug starts and outcome attainment within 70 weeks of diagnosis were compared with Kaplan Meier survival analysis and multivariable Cox regression. RESULTS: The 2005-2010 and 2017-2021 cohorts included 1128 and 721 patients, respectively. JIA category distribution and baseline clinical juvenile idiopathic arthritis disease activity (cJADAS10) scores at enrolment were comparable. By 70 weeks, 6% of patients (95% CI 5, 7) in the 2005-2010 and 26% (23, 30) in the 2017-2021 cohort had started a biologic DMARD (bDMARD), and 43% (40, 47) and 60% (56, 64) had started a conventional DMARD (cDMARD), respectively. Outcome attainment was 64% (61, 67) and 83% (80, 86) for Inactive disease (Wallace criteria), 69% (66, 72) and 84% (81, 87) for minimally active disease (cJADAS10 criteria), 57% (54, 61) and 63% (59, 68) for pain control (<1/10), and 52% (47, 56) and 54% (48, 60) for a good health-related quality of life. CONCLUSION: Although baseline disease characteristics were comparable in the 2005-2010 and 2017-2021 cohorts, cDMARD and bDMARD use increased with a concurrent increase in minimally active and inactive disease. Improvements in parent and patient reported outcomes were smaller than improvements in disease activity.

Cardiovascular outcomes in children with Kawasaki disease: a population-based cohort study.

BACKGROUND: The risk of cardiovascular events after Kawasaki disease (KD) remains uncertain. Our objective was to determine the risk of cardiovascular events and mortality after KD. METHODS: Population-based retrospective cohort study using Ontario health administrative databases (0-18 years; 1995-2018). EXPOSURE: pediatric KD hospitalizations. Each case was matched to 100 non-exposed controls. PRIMARY OUTCOME: major adverse cardiac events (MACE; cardiovascular death, myocardial infarction, or stroke composite). SECONDARY OUTCOMES: composite cardiovascular events and mortality. We determined incidence rates and adjusted hazard ratios (aHR) using multivariable Cox models. RESULTS: Among 4597 KD survivors, 79 (1.7%) experienced MACE, 632 (13.8%) composite cardiovascular events, and 9 (0.2%) died during 11-year median follow-up. The most frequent cardiovascular events among KD survivors were ischemic heart disease (4.6 events/1000 person-years) and arrhythmias (4.5/1000 person-years). KD survivors were at increased risk of MACE between 0-1 and 5-10 years, and composite cardiovascular events at all time periods post-discharge. KD survivors had a lower mortality risk throughout follow-up (aHR 0.36, 95% CI 0.19-0.70). CONCLUSION: KD survivors are at increased risk of post-discharge cardiovascular events but have a lower risk of death, which justifies enhanced cardiovascular disease surveillance in these patients. IMPACT: Among 4597 Kawasaki disease (KD) survivors, 79 (1.7%) experienced major adverse cardiac events (MACE) and 632 (13.8%) had composite cardiovascular events during 11-year median follow-up. KD survivors had significantly higher risks of post-discharge MACE and cardiovascular events versus non-exposed children. Only nine KD survivors (0.2%) died during follow-up, and the risk of mortality was significantly lower among KD survivors versus non-exposed children. Childhood KD survivors should receive preventative counseling and cardiovascular surveillance, aiming to mitigate adult cardiovascular disease.

A survey of Canadian adult rheumatologists' knowledge, comfort level, and barriers in assessing psychosocial needs of young adults with rheumatic diseases.

To assess adult rheumatologists' comfort level, current practices, and barriers to provision of optimal care in supporting young adults with pediatric-onset rheumatic conditions in Canada. Survey questions were informed by literature review, a needs assessment, and using milestones listed by the Royal College of Physicians and Surgeons of Canada for the entrustable professional activities (EPAs) applicable to care for rheumatology patients transitioning to adult practice. The electronic survey was distributed to adult rheumatology members of the Canadian Rheumatology Association over 4 months. Four hundred and fifty-one rheumatologists received the survey, with a response rate of 15.2%. Most respondents were from Ontario and had been in practice ≥ 10 years. Three quarters reported a lack of training in transition care although the same proportion were interested in learning more about the same. Approximately 40% felt comfortable discussing psychosocial concerns such as gender identity, sexuality, contraception, drug and alcohol use, vaping, and mental health. Despite this, 45-50% reported not discussing vaping or gender identity at all. The most frequently reported barriers to providing transition care were lack of primary care providers, allied health support, and training in caring for this age group. Most adult rheumatologists lack formal training in transition care and view it as a barrier to providing care for this unique patient population. Future educational initiatives for adult rheumatology trainees should include issues pertaining to adolescents and young adults. More research is needed to assess the effectiveness of resources such as transition navigators in ensuring a successful transition process.

Cardiovascular Outcomes During Index Hospitalization in Children with Kawasaki Disease in Ontario, Canada.

Kawasaki disease (KD) is a common childhood vasculitis associated with coronary artery aneurysms (CAA). However, there is limited published data on other cardiovascular events diagnosed during acute KD hospitalizations. Our objectives were to determine the incidence of cardiovascular events during acute KD hospitalizations, stratified by age at admission, CAA status, and pediatric intensive care unit (PICU) admission status. We identified all children (0-18 year) hospitalized with a new KD diagnosis in Ontario, between 1995 and 2018, through validated algorithms using population health administrative databases. We excluded children previously diagnosed with KD and non-Ontario residents. We evaluated for cardiovascular events that occurred during the acute KD hospitalizations, defined by administrative coding. Among 4597 children hospitalized with KD, 3307 (71.9%) were aged 0-4 years, median length of stay was 3 days (IQR 2-4), 113 children (2.5%) had PICU admissions, and 119 (2.6%) were diagnosed with CAA. During acute hospitalization, 75 children were diagnosed with myocarditis or pericarditis (1.6%), 47 with arrhythmias (1.0%), 25 with heart failure (0.5%), and ≤ 5 with acute MI (≤ 0.1%). Seven children underwent cardiovascular procedures (0.2%). Older children (10-18 years), children with CAA, and children admitted to the PICU were more likely to experience cardiovascular events, compared with children aged 0-4 years, without CAA or non-PICU admissions, respectively. The frequency of non-CAA cardiovascular events during acute KD hospitalizations did not change significantly between 1995 and 2018. During acute KD hospitalizations, older children, children with CAA, and PICU admissions are at higher risk of cardiovascular complications, justifying closer monitoring of these high-risk individuals.

Mind the Gap: A Longitudinal Analysis of the Experiences of Adolescents and Parents in a Rheumatology Transition Clinic.

BACKGROUND: The transition from pediatric to adult care is a critical time when adolescent patients and their families face many challenges. This period can be associated with an increase in disease-related morbidity and mortality. The aim of our study is to identify gaps in transition-related care to help guide areas for improvement. METHODS: Patients (14-19 years) with juvenile idiopathic arthritis or systemic lupus erythematosus and one of their parents were recruited from the McMaster Rheumatology Transition Clinic. Both were asked to complete the Mind the Gap questionnaire, a validated tool to assess experience and satisfaction with transition care in a clinic setting. The questionnaire, addressing 3 important domains of care: management of the environment, provider characteristics, and process issues, was completed twice-once based on their current clinical experience and again based on their ideal clinical encounter. Positive scores suggest current care is less than ideal; negative scores suggest current care exceeds the ideal experience. RESULTS: Most patients (n = 65, 68% female) had a diagnosis of juvenile idiopathic arthritis (87%). Patients identified mean gap scores between 0.2 and 0.3 for each domain of Mind the Gap, with female patients having higher gap scores compared with male patients. Parents (n = 51) identified gap scores between 0.0 and 0.3. Patients identified process issues as having the largest gap, whereas parents identified management of the environment as having the largest gap. CONCLUSIONS: We identified several gaps in transition clinic care relative to what patients and parents identify as ideal. These can be used to improve the rheumatology transition care that is currently being provided.

Self-Reported Transition Readiness of Adolescent Patients with Rheumatic Disease: Do the Parents Agree?

Our objective was to compare transition readiness assessment scores from adolescents with rheumatic disease with their parents and analyze their level of agreement. We found that adolescents and parents generally agree on the level of the transition readiness; however, there is occasional disagreement in specific domains.

Incidence of chronic immune-mediated inflammatory diseases after diagnosis with Kawasaki disease: a population-based cohort study.

OBJECTIVES: Kawasaki disease (KD) is an immune-mediated vasculitis of childhood with multi-organ inflammation. We determined the risk of subsequent immune-mediated inflammatory disease (IMID), including arthritis, type 1 diabetes, IBD, autoimmune liver disease, primary sclerosing cholangitis and multiple sclerosis. METHODS: We conducted a matched population-based cohort study using health administrative data from Ontario, Canada. Children aged <18 years born between 1991 and 2016 diagnosed with KD (n = 3753) were matched to 5 non-KD controls from the general population (n = 18 749). We determined the incidence of IMIDs after resolution of KD. Three- and 12-month washout periods were used to exclude KD-related symptoms. RESULTS: There was an elevated risk of arthritis in KD patients compared with non-KD controls, starting 3 months after index date [103.0 vs 12.7 per 100 000 person-years (PYs); incidence rate ratio 8.07 (95% CI 4.95, 13.2); hazard ratio 8.08 (95% CI 4.95, 13.2), resulting in the overall incidence of IMIDs being elevated in KD patients (175.1 vs 68.0 per 100 000 PYs; incidence rate ratio 2.58 (95% CI 1.93, 3.43); hazard ratio 2.58, 95% CI 1.94, 3.43]. However, there was no increased risk for diabetes, IBD, autoimmune liver disease, primary sclerosing cholangitis or multiple sclerosis in KD patients. Similar results were observed using a 12-month washout period. CONCLUSION: Children diagnosed with KD were at increased risk of arthritis following the acute KD event, but not other IMIDs. Health-care providers should monitor for arthritis in children following a diagnosis of KD.

Impact of the COVID-19 pandemic on juvenile idiopathic arthritis presentation and research recruitment: results from the CAPRI registry.

OBJECTIVE: The COVID-19 pandemic has disrupted healthcare delivery and clinical research worldwide, with data from areas most affected demonstrating an impact on rheumatology care. This study aimed to characterize the impact of the pandemic on the initial presentation of JIA and JIA-related research in Canada. METHODS: Data collected from the Canadian Alliance of Pediatric Rheumatology Investigators JIA Registry from the year pre-pandemic (11 March 2019 to 10 March 2020) was compared with data collected during the first year of the pandemic (11 March 2020 to 10 March 2021). Outcomes included time from symptom onset to first assessment, disease severity at presentation and registry recruitment. Proportions and medians were used to describe categorical and continuous variables, respectively. RESULTS: The median time from symptom onset to first assessment was 138 (IQR 64-365) days pre-pandemic vs 146 (IQR 83-359) days during the pandemic. The JIA category frequencies remained overall stable (44% oligoarticular JIA pre-pandemic, 46.8% pandemic), except for systemic JIA (12 cases pre-pandemic, 1 pandemic). Clinical features, disease activity (cJADAS10), disability (CHAQ) and quality of life (JAQQ) scores were similar between the two cohorts. Pre-pandemic, 225 patients were enrolled, compared with 111 in the pandemic year, with the greatest decrease from March to June 2020. CONCLUSIONS: We did not observe the anticipated delay in time to presentation or increased severity at presentation, suggesting that, within Canada, care adapted well to provide support to new patient consults without negative impacts. The COVID-19 pandemic was associated with an initial 50% decrease in registry enrolment but has since improved.

Health care utilization and costs following Kawasaki disease.

Objectives: Kawasaki disease (KD) is a common childhood vasculitis with increasing incidence in Canada. Acute KD hospitalizations are associated with high health care costs. However, there is minimal health care utilization data following initial hospitalization. Our objective was to determine rates of health care utilization and costs following KD diagnosis. Methods: We used population-based health administrative databases to identify all children (0 to 18 years) hospitalized for KD in Ontario between 1995 and 2018. Each case was matched to 100 nonexposed comparators by age, sex, and index year. Follow-up continued until death or March 2019. Our primary outcomes were rates of hospitalization, emergency department (ED), and outpatient physician visits. Our secondary outcomes were sector-specific and total health care costs. Results: We compared 4,597 KD cases to 459,700 matched comparators. KD cases had higher rates of hospitalization (adjusted rate ratio 2.07, 95%CI 2.00 to 2.15), outpatient visits (1.30, 95%CI 1.28 to 1.33), and ED visits (1.22, 95%CI 1.18 to 1.26) throughout follow-up. Within 1 year post-discharge, 717 (15.6%) KD cases were re-hospitalized, 4,587 (99.8%) had ≥1 outpatient physician visit and 1,695 (45.5%) had ≥1 ED visit. KD cases had higher composite health care costs post-discharge (e.g., median cost within 1 year: $2466 CAD [KD cases] versus $234 [comparators]). Total health care costs for KD cases, respectively, were $13.9 million within 1 year post-discharge and $54.8 million throughout follow-up (versus $2.2 million and $23.9 million for an equivalent number of comparators). Conclusions: Following diagnosis, KD cases had higher rates of health care utilization and costs versus nonexposed children. The rising incidence and costs associated with KD could place a significant burden on health care systems.

Incidence and short-term outcomes of Kawasaki disease.

OBJECTIVE: Kawasaki disease (KD) is a childhood vasculitis with conflicting reported North American trends in incidence and patient characteristics. OBJECTIVES: (1) determine KD incidence between 1995 and 2017; (2) compare patient characteristics by era and age group; (3) determine complication and cardiovascular follow-up rates. METHODS: We used population-based health administrative data to identify children (0-18 yr) hospitalized with KD in Ontario, Canada between 1995 and 2017. We excluded children with prior KD diagnosis or incomplete records. We determined the annualized incidence and follow-up trends. RESULTS: KD was diagnosed in 4,346 children between 1995 and 2017. Annual KD incidence was 22.0 (<5 yr), 6.1 (5-9 yr), and 0.6 (10-18 yr) per 100,000 children. KD incidence increased significantly for all age groups, including from 18.4 to 25.0 cases per 100,000 children <5 yr. Ninety-day mortality occurred in ≤5 children (≤0.1%). Coronary artery aneurysm (CAA) occurred in 106 children (2.4%, 95% confidence interval 2.0-2.9) during admission and 151 (3.5%, 95% confidence interval 3.0-4.1) during 11-year median follow-up. Children 10-18 yr had longer hospitalizations (4.3 vs. 3.5 days, p = 0.003) and more CAA (7.4% vs. 3.4%, p = 0.007). By 1-year post-diagnosis, 3970 (91.3%) and 2576 (59.3%) children had echocardiography and cardiology follow-up, respectively. CONCLUSIONS: KD incidence is increasing in Ontario, with greater healthcare utilization from hospitalizations and subsequent follow-up. IMPACT: 4346 children were hospitalized for Kawasaki disease over 22 years in Ontario, and Kawasaki disease incidence increased significantly for all age groups, males and females. Older children (10-18 years) had longer hospital length of stay, more PICU admissions and more frequent coronary artery aneurysms. Nearly all children with Kawasaki disease had follow-up echocardiography within 1 year.

Pediatric Patients with a Dual Diagnosis of Inflammatory Bowel Disease and Chronic Recurrent Multifocal Osteomyelitis.

ABSTRACT: There is a paucity of information about the epidemiology, pathophysiology, and treatment of patients with a dual diagnosis of inflammatory bowel disease (IBD) and chronic recurrent multifocal osteomyelitis (CRMO). A retrospective chart review was performed of patients at McMaster Children's Hospital with a diagnosis of either IBD or CRMO, to identify those with the dual diagnosis over a 10-year period. A dual diagnosis was identified in seven patients. Most patients (6/7) had a diagnosis of IBD first and were subsequently diagnosed with CRMO. At the time of CRMO diagnosis, IBD treatment regimens included one or more of, sulfasalazine (1/6), infliximab (3/6), adalimumab (1/6), or no treatment (1/6). Although the etiology of the link remains unknown, there does not seem to be an association to a specific IBD subtype, age, or treatment. Our patient population demonstrated a response to biologic agents, specifically tumor necrosis factor-α inhibitors, as treatment for both conditions.

Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience.

OBJECTIVE: To assess long-term outcomes of children with JIA diagnosed in the biologic era. METHODS: Chart review of patients prospectively enrolled in the Research in Arthritis in Canadian Children Emphasizing Outcomes inception cohort at two Canadian centres. Inactive disease and remission were defined according to Wallace criteria. RESULTS: We included 247 of 254 (97%) eligible patients diagnosed 2005-10. At the last follow-up visit at a median age of 16.9 years, 47% were in remission off medications, 25% in remission on medications and 27% had active disease; 51% were on at least one anti-rheumatic medication (22% on biologics). Patients with systemic JIA had the highest frequency of remission off medications (70%) and patients with RF-positive polyarthritis had the lowest (18%) (P <0.05 by Fisher's exact test). Among 99 patients with oligoarthritis at enrolment, 14 (14%) had an oligoarthritis extended course. Forty-five patients (18%) had at least one erosion or joint space narrowing in X-rays or MRI, and two (0.8%) required joint replacement. CONCLUSION: Relative to historical cohorts, this study suggests a reduction in JIA permanent damage, a more favourable prognosis for systemic JIA and a lower progression to oligoarthritis extended category. However, in an era of biologic therapy, one in four patients with JIA still enter adulthood with active disease and one in two still on treatment.

A new Canadian inception cohort for juvenile idiopathic arthritis: The Canadian Alliance of Pediatric Rheumatology Investigators Registry.

OBJECTIVES: The aim was to describe the design, methods and initial findings of a new Canadian inception cohort of children with JIA, The Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) JIA Registry. METHODS: The CAPRI JIA Registry was started in 2017 to collect information prospectively on children enrolled within 3 months of JIA diagnosis across Canada. The registry has a non-traditional modular design, with no artificially set times for registry visits to occur, streamlined multi-method data collection that requires 2-4 min per visit, and reports cumulative incidence of treatments, outcomes and adverse events calculated by Kaplan-Meier survival methods. RESULTS: A total of 166 patients, enrolled a median of 6 weeks after JIA diagnosis at 10 centres, were included. The median age at diagnosis was 9 years [interquartile range (IQR) 3, 13], 61% were female and 51% had oligoarticular JIA. The median three-variable clinical Juvenile Arthritis Disease Activity Score was 6.5 (IQR 4, 10) at enrolment, and the median time to first attainment of clinically inactive disease (CID) was 24 weeks (by 1 year, 81%). Within 1 year of diagnosis, 70% of patients had started a DMARD and 35% a biologic agent. The rates of adverse events and serious adverse events were 60 and 5.8 per 100 patient-years, respectively. CONCLUSION: This streamlined and flexible registry minimizes the burden of data collection and interference with clinic operations. Initial findings suggest that treatments for newly diagnosed patients with JIA in Canada have intensified, and now 81% of patients attain CID within 1 year of diagnosis.

Organizational Learning in the Morbidity and Mortality Conference.

INTRODUCTION: The focus of morbidity and mortality conferences (M&MCs) has shifted to emphasize quality improvement and systems-level care. However, quality improvement initiatives targeting systems-level errors are challenged by learning in M&MCs, which occurs at the individual attendee level and not at the organizational level. Here, we aimed to describe how organizational learning in M&MCs is optimized by particular organizational and team cultures. METHODS: A prospective, multiple-case study design was used. Using purposive sampling, three cases covering different medical/surgical specialties in North America were chosen. Data collection included direct observations of the M&MC, semistructured interviews with key M&MC members, and documentary information. RESULTS: The role of the M&MC in all cases integrated two key concepts: recognition of system-wide trends and learning from error, at an organizational and team level. All cases provided evidence of double-loop learning and used organizational memory strategies to ensure knowledge was retained within the organization. A patient safety culture was linked to the promotion of open communication, fostering learning from adverse events. CONCLUSION: This study describes three cases of systems-oriented M&MCs that reflected elements of organizational learning theory. The M&MC can therefore provide a context for organizational learning, allowing optimal learning from adverse events.

Asymptomatic intraspinal epidermoid cyst in a 7-year-old male with juvenile idiopathic arthritis identified by an advanced physiotherapist practitioner: a case report.

BACKGROUND: Pediatric intraspinal epidermoid cysts are rare with potential to cause life-altering outcomes if not addressed. Reports to date describe symptomatic presentations including loss of bladder or bowel function and motor and sensory losses. This case report identifies the diagnostic challenge of an asymptomatic intraspinal epidermoid cyst in the cauda equina region presenting in a 7-year-old male with juvenile idiopathic arthritis (JIA). DIAGNOSIS: An advanced physiotherapist practitioner assessed and diagnosed a previously healthy 7-year-old-male of South Asian descent with JIA based on persistent knee joint effusions. Complicating factors delayed the investigation of abnormal functional movement patterns, spinal and hip rigidity and severe restriction of straight leg raise, all atypical for JIA. Further delaying the diagnosis was the lack of subjective complaints including no pain, no reported functional deficits, and no neurologic symptoms. A spinal MRI investigation 10-months from initial appointment identified intraspinal epidermoid cysts occupying the cauda equina region requiring urgent referral to neurosurgery. DISCUSSION: Clinical characteristics and pattern recognition are essential for diagnosing spinal conditions in pediatric populations. Diagnostic challenges present in this case included co-morbidity (JIA), a severe adverse reaction to treatment, a lack of subjective complaints and a very low prevalence of intraspinal epidermoid cysts. IMPACT STATEMENTS: Early signs of pediatric asymptomatic intraspinal epidermoid cysts included abnormal functional movement patterns, rigidity of spine, severely limited straight leg raise and hip flexion without pain. Advanced physiotherapist practitioners can be integral to pediatric rheumatology teams considering their basic knowledge in musculoskeletal examination and functional mobility assessment when identifying rare spinal conditions that present within the complex context of rheumatic diseases.

Relationship of Fatigue, Pain Interference, and Physical Disability in Children Newly Diagnosed With Juvenile Idiopathic Arthritis.

OBJECTIVE: Our objectives were to quantify the relationships among fatigue, pain interference, and physical disability in children with juvenile idiopathic arthritis (JIA) and to test whether fatigue mediates the relationship between pain interference and physical disability in JIA. METHODS: Patients enrolled within three months of JIA diagnosis in the Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) Registry between February 2017 and May 2023 were included. Their parents completed the Patient-Reported Outcomes Measurement Information System fatigue and pain interference short proxy questionnaires and the Childhood Health Assessment Questionnaire disability index at registry enrollment. Associations were assessed using Pearson correlations and multiple linear regression. Structural equation modeling (SEM) was used to test if fatigue mediates the relationship between pain interference and physical disability. RESULTS: Among 855 patients (61.4% female, 44.1% with oligoarthritis), most reported fatigue and pain interference scores similar to those in the reference population, but 15.6% reported severe fatigue and 7.3% reported severe pain interference, with wide variation across JIA categories. Fatigue was strongly correlated with pain interference (r = 0.72, P < 0.001) and with physical disability (r = 0.60, P < 0.001). Pain interference (ß = 0.027, P < 0.001) and fatigue (ß = 0.013, P < 0.001) were both associated with physical disability after controlling for each other and potential confounders. SEM supported our hypothesis that fatigue partially mediates the relationship between pain interference and physical disability. CONCLUSION: Our findings suggest both fatigue and pain interference are independently associated with physical disability in children newly diagnosed with JIA, and the effect of pain interference may be partly mediated by fatigue.