COVID-19 vaccination safety and associated health care utilization among adults with inflammatory bowel disease - a population-based self-controlled case series analysis.

BACKGROUND AND AIMS: There is an incomplete understanding of the full safety profiles of repeated COVID-19 vaccinations in patients with inflammatory bowel disease (IBD). Among individuals with IBD, we assessed whether COVID-19 vaccines were associated with serious adverse events of special interest (AESI) and health care utilization [all-cause hospitalizations, Emergency Department (ED) visits, gastroenterology visits, IBD-related visits]. METHODS: Using comprehensive administrative health data from Ontario, Canada, adults with IBD who received at least one COVID-19 vaccine from December 2020-January 2022 were included. Self-controlled case series analyses were conducted to evaluate the relative incidence rates of AESI and health care utilization outcomes across post-vaccination risk and control periods. RESULTS: Among 88,407 IBD patients, 99.7% received mRNA vaccines and 75.9% received ≥ 3 doses. Relative to control periods, we did not detect an increase in AESI. IBD patients had fewer all-cause hospitalizations during post-vaccination risk periods. Patients experienced more all-cause ED visits after dose 2 [Relative Incidence (RI):1.08(95%CI:1.04-1.12)] but fewer visits after doses 3 [RI:0.85 (95%CI:0.81-0.90)] and 4 [RI:0.73 (95%CI:0.57-0.92)]. There was no increase in gastroenterologist visits or IBD-related health care utilization post-vaccination. There were fewer IBD-related hospitalizations after dose 1 [RI:0.84 (95%CI:0.72-0.98)] and 3 [RI:0.63 (95%CI:0.52-0.76)], fewer IBD-related ED visits after dose 3 [RI:0.81 (95%CI:0.71-0.91)] and 4 [RI:0.55 (95%CI:0.32-0.96)], and fewer outpatient visits after dose 2 [RI:0.91 (95%CI:0.90-0.93)] and 3 [RI:0.87 (95%CI:0.86-0.89)]. CONCLUSION: This population-based study did not detect increased AESI, all-cause or IBD-related health care utilization following COVID-19 vaccination, suggesting a lack of association between vaccination and increased disease activity.

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.

Evaluating the time-varying risk of hypertension, cardiac events, and mortality following Kawasaki disease diagnosis.

BACKGROUND: This study evaluated the risk of hypertension, major adverse cardiac events (MACE), and all-cause mortality in Kawasaki disease (KD) patients up to young adulthood. METHODS: An inception cohort of 1169 KD patients between 1991 and 2008 from a tertiary-level hospital in Ontario, Canada was linked with health administrative data to ascertain outcomes up to 28 years of follow-up. Their risk was compared with 11,690 matched population comparators. The primary outcome was hypertension and secondary outcomes were MACE and death. RESULTS: After a median follow-up of 20 years [IQR: 8.3], the cumulative incidence of hypertension and MACE in the KD group was 3.8% (95% CI: 2.5-5.5) and 1.2% (95% CI: 0.6-2.4%), respectively. The overall survival probability in the KD group was 98.6% (95% CI: 97.2-99.3%). Relative to comparators, KD patients were at an increased risk for hypertension [aHR: 2.2 (95% CI: 1.5-3.4)], death [aHR: 2.5 (95% CI: 1.3-5.0)], and MACE [aHR: 10.7 (95% CI: 6.4-17.9)]. For hypertension and MACE, the aHR was the highest following diagnosis and then the excess risk diminished after 16 and 13 years of follow-up, respectively. MACE occurred largely in KD patients with coronary aneurysms [cumulative incidence: 12.8%]. CONCLUSIONS: KD patients demonstrated a reassuring cardiac prognosis up to young adulthood with low events and excellent survival. KD patients were at increased risk for hypertension, but this excess risk occurred early and declined with time. IMPACT: With the current standard of care, KD patients demonstrated favorable cardiac prognosis, with low events of hypertension, MACE, and excellent survival. Hypertension and MACE risk appear to be highest around the time of KD diagnosis. MACE occurred primarily in KD patients with coronary aneurysms. Our findings are reassuring to KD patients, families, and their providers. Our study demonstrated an association between KD exposure and hypertension. This association is relatively novel. Previous studies have remained conflicting if KD contributes to long-term atherosclerotic risk.

A hot spot for systemic lupus erythematosus, but not for psoriatic arthritis, identified by spatial analysis suggests an interaction between ethnicity and place of residence.

OBJECTIVE: To describe the spatial distribution of incident cases of systemic lupus erythematosus (SLE) using geographic information systems (GIS). METHODS: Spatial analyses were carried out on 890 SLE patients and 541 psoriatic arthritis (PsA) patients (controls). Age- and sex-adjusted rates for SLE/PsA for each census tract were calculated using denominator population values from the Canadian census. Spatial variations in relative risk were estimated by modeling risk as the product of a time effect, an age effect, and a spatially autocorrelated risk surface to identify hot spots. Patients within the detected hot spot were compared to those outside the hot spot to identify explanatory factors. RESULTS: SLE patients were predominantly female (87.75%) and the incidence rate was highest among those 15-19 years of age (2.4 cases/100,000 person-years). In an SLE hot spot containing 59 patients, 100% of the patients were female and 49.1% (n = 29) were Caucasian, while outside of the hot spot, 86.9% (n = 722) of the patients were female and 68.4% (n = 568) were Caucasian. The proportion of cases of Chinese ethnicity was significantly greater within the hot spot. An interaction was found between Chinese ethnicity and residence within the hot spot, with the risk of SLE to the Chinese population found to be twice the risk to the non-Chinese population. CONCLUSION: GIS was used to map SLE cases and a hot spot was identified after adjustment for age and sex. Ethnicity by itself did not confer an increased risk of SLE, but the interaction of ethnicity with location of residence significantly increased the risk of SLE.

Safety and Health Care Use Following COVID-19 Vaccination Among Adults With Rheumatoid Arthritis: A Population-Based Self-Controlled Case Series Analysis.

OBJECTIVE: To determine if coronavirus disease 2019 (COVID-19) vaccines were associated with adverse events of special interest (AESIs) and healthcare use among adults with rheumatoid arthritis (RA). METHODS: Among adults with RA who received at least 1 COVID-19 vaccine, a self-controlled case series (SCCS) analysis was conducted to evaluate relative incidence (RI) rates of AESIs (Bell palsy, idiopathic thrombocytopenia, acute disseminated encephalomyelitis, pericarditis/myocarditis, Guillain-Barré syndrome, transverse myelitis, myocardial infarction, anaphylaxis, stroke, deep vein thrombosis, pulmonary embolism, narcolepsy, appendicitis, and disseminated intravascular coagulation) in any 21-day period following vaccination compared to control periods. Secondary outcomes included emergency department (ED) visits, hospitalizations, and rheumatology visits. A matched non-RA comparator group was created and a separate SCCS analysis was conducted. RI ratios (RIRs) were used to compare RA and non-RA groups. RESULTS: Among 123,466 patients with RA and 493,864 comparators, the majority received mRNA vaccines. For patients with RA, relative to control periods, AESIs were not increased. ED visits increased after dose 2 (RI 1.06, 95% CI 1.03-1.10) and decreased after dose 3 (RI 0.93, 95% CI 0.89-0.96). Hospitalizations were lower after the first (RI 0.83, 95% CI 0.78-0.88), second (RI 0.86, 95% CI 0.81-0.92), and third (RI 0.89, 95% CI 0.83-0.95) doses. Rheumatology visits increased after dose 1 (RI 1.08, 95% CI 1.07-1.10), and decreased after doses 2 and 3. Relative to comparators, patients with RA had a higher AESI risk after dose 3 (RIR 1.28, 95% CI 1.05-1.56). Patients with RA experienced fewer ED visits (RIR 0.73, 95% CI 0.58-0.90) and hospitalizations (RIR 0.52, 95% CI 0.36-0.75) after dose 4. CONCLUSION: COVID-19 vaccines in patients with RA were not associated with an increase in AESI risk or healthcare use after every dose.

The Effects of Continuity of Rheumatology Care on Emergency Department Utilization and Hospitalizations for Individuals With Early Rheumatoid Arthritis: A Population-Based Study.

OBJECTIVE: To determine if continuity of rheumatology care influences rates of emergency department (ED) visits and hospitalizations in patients with rheumatoid arthritis (RA). METHODS: A closed inception cohort of patients with RA diagnosed between 2000 and 2009 were followed until December 31, 2019. During the first 5 years following diagnosis, we categorized patients into 3 rheumatology care continuity groups (high, intermediate, and not retained in rheumatology care). Using a landmark analysis, we compared rates of ED visits and hospitalizations during follow-up. Multivariable Poisson regression models were used to estimate rate ratios (RRs), adjusting for demographics, comorbidities, and health services access and supply measures. RESULTS: The cohort included 38,528 patients, of which 57.7% (n = 22,221) were classified in the high rheumatology continuity group, 17.2% (n = 6636) were in the intermediate group, and 25.1% (n = 9671) were not retained in rheumatology care. Relative to the high continuity group, both the intermediate and nonretention groups had higher ED rates (RR 1.14, 95% CI 1.08-1.20, and RR 1.12, 95% CI 1.08-1.16, respectively). The intermediate group also experienced higher adjusted hospitalization rates (207.4, 95% CI 203.0-211.8 per 1000 person-years [PY]) than the high continuity group (193.5, 95% CI 191.4-195.6 per 1000 PY). CONCLUSION: Patients with RA with higher continuity of rheumatology care had lower rates of ED visits and hospitalizations compared to those who did not receive continuous rheumatology care during the first 5 years of follow-up. These findings provide evidence to support the value of early and continuous rheumatology care for reducing hospitalizations and ED visits.

An Overview of Reviews to Inform Organization-Level Interventions to Address Burnout in Rheumatologists.

OBJECTIVE: To identify organization-directed strategies that could be implemented to prevent burnout among rheumatologists. METHODS: A search of English language articles published 2011 or later was conducted on Cochrane Database of Systematic Reviews, Embase, Medline, and PsycInfo on January 25, 2022. Included reviews had ≥ 1 primary studies with ≥ 10% of participants who were physicians, recorded burnout as an outcome, and described an organization-directed intervention to prevent burnout. Overlap of primary studies across reviews was assessed. The final review inclusion was determined by study quality, minimization of overlap, and maximization of intervention breadth. The A Measurement Tool to Assess Systematic Reviews (AMSTAR) 2 tool was used for quality assessment. Included studies and interventions were assessed by rheumatologists for their applicability to rheumatology. RESULTS: A total of 17 reviews, including 15 systematic reviews, 1 realist review, and 1 umbrella review were included. AMSTAR 2 quality ratings classified 5 systematic reviews as low quality, 1 as moderate, and 9 as critically low. There was significant heterogeneity between and within reviews. Six conducted a metaanalysis and 11 provided a qualitative summary of findings. The following intervention types were identified as having possible applicability to rheumatology: physician workflow and organizational strategies; peer support and formal communication training; leadership support; and addressing stress, mental health, and mindfulness. Across interventions, mindfulness had the highest quality of evidence to support its effectiveness. CONCLUSION: Although the quality of evidence for interventions to prevent burnout in physicians is low, promising strategies such as mindfulness have been identified.

Choosing Wisely: The Canadian Rheumatology Association Pediatric Committee's List of Items Physicians and Patients Should Question.

OBJECTIVE: To develop a list of tests or treatments frequently used in pediatric rheumatology practice that may be unnecessary based on existing evidence. METHODS: A Choosing Wisely (CW) working group composed of 16 pediatric rheumatologists, 1 allied health professional, 1 parent, and 1 patient used the Delphi method to generate, rank, and refine a list of tests and treatments that may be unnecessary or harmful. The items with the highest content agreement and perceived impact were presented in a survey to all Canadian Rheumatology Association (CRA) physicians who practice pediatric rheumatology. Respondents were asked to rate their agreement and impact, and to rank the items. Five items with the highest composite scores and 2 additional items selected by the CW working group were put forward for literature review. RESULTS: The initial Delphi procedure generated 80 items. After 3 rounds, the list was narrowed to 13 items. The survey was completed by 41/81 (51%) CRA pediatric members across Canada. Respondent characteristics were similar to those of the CRA pediatric membership for self-reported gender, geographical location, and career stage. The highest composite score items were antinuclear antibody testing, drug toxicity monitoring, HLA-B27 testing, rheumatoid factor/anticyclic citrullinated peptide testing, and Lyme serology testing. Two additional items (numerous or repeated intraarticular corticosteroid injections, and autoinflammatory diseases genetic testing) were also selected. Literature review was performed for these 7 highest priority items. CONCLUSION: We have identified areas for quality improvement in the evaluation and treatment of rheumatic diseases in Canadian children.

The Long-term Cardiac and Noncardiac Prognosis of Kawasaki Disease: A Systematic Review.

CONTEXT: It is uncertain if children with Kawasaki Disease (KD) are at risk for non-cardiac diseases and if children with KD but without coronary artery aneurysms (CAA) are at risk for long-term cardiac complications. OBJECTIVE: To determine the long-term mortality and prognosis of children after KD. DATA SOURCES: Medline, Embase, and the Cochrane Central Register. STUDY SELECTION: Controlled trials and observational studies were included if they included children with KD and reported mortality, major adverse cardiovascular events (MACE), chronic cardiac or other disease over an average follow-up of ≥1 year. DATA EXTRACTION: Data extracted included sample size, age at diagnosis, the proportion with coronary artery aneurysms (CAA), follow-up duration, and outcome(s). RESULTS: Seventy-four studies were included. Thirty-six studies reported mortality, 55 reported a cardiac outcome, and 12 reported a noncardiac outcome. Survival ranged from 92% to 99% at 10 years, 85% to 99% at 20 years, and 88% to 94% at 30 years. MACE-free survival, mostly studied in those with CAA, varied from 66% to 91% at 10 years, 29% to 74% at 20 years, and 36% to 96% at 30 years. Seven of 10 studies reported an increased risk in early atherosclerosis. All 6 included studies demonstrated an increased risk in allergic diseases. LIMITATIONS: Our study may have missed associated chronic comorbidities because short-term studies were excluded. The majority of outcomes were evaluated in East-Asian patients, which may limit generalizability. Studies frequently excluded patients without CAA and did not compare outcomes to a comparison group. CONCLUSIONS: Studies demonstrate >90% survival up to 30 years follow-up. MACE is observed in children with CAA, but is not well studied in those without CAA.

Variations in Pediatric Rheumatology Workforce and Care Processes Across Canada.

OBJECTIVE: To examine the Canadian pediatric rheumatology workforce and care processes. METHODS: Pediatric rheumatologists and allied health professionals (AHPs) participated. A designee from each academic center provided workforce information including the number of providers, total and breakdown of full-time equivalents (FTEs), and triage processes. We calculated the clinical FTE (cFTE) available per 75,000 (recommended benchmark) and 300,000 (adjusted) children using 2019 census data. The national workforce deficit was calculated as the difference between current and expected cFTEs. Remaining respondents were asked about ambulatory practices. RESULTS: The response rate of survey A (workforce information) and survey B (ambulatory practice information) was 100% and 54%, respectively. The majority of rheumatologists (91%) practiced in academic centers. The median number of rheumatologists per center was 3 (IQR 3) and median cFTE was 1.9 (IQR 1.5). The median cFTE per 75,000 was 0.2 (IQR 0.3), with a national deficit of 80 cFTEs. With the adjusted benchmark, there was no national deficit, but there was a regional maldistribution of rheumatologists. All centers engaged in multidisciplinary practices with a median of 4 different AHPs, although the median FTE for AHPs was ≤ 1. Most centers (87%) utilized a centralized triage process. Of 9 (60%) centers that used an electronic triage process, 6 were able to calculate wait times. Most clinicians integrated quality improvement practices, such as previsit planning (67%), postvisit planning (68%), and periodic health outcome monitoring (36-59%). CONCLUSION: This study confirms a national deficit at the current recommended benchmark. Most rheumatologists work in multidisciplinary teams, but AHP support may be inadequate.

A Comparison of International League of Associations for Rheumatology and Pediatric Rheumatology International Trials Organization Classification Systems for Juvenile Idiopathic Arthritis Among Children in a Canadian Arthritis Cohort.

OBJECTIVE: The aim of the Paediatric Rheumatology International Trials Organisation (PRINTO) juvenile idiopathic arthritis (JIA) classification criteria, which is still in development, is to identify homogeneous groups of JIA patients. This study was undertaken to compare International League of Associations for Rheumatology (ILAR) JIA classification criteria and PRINTO JIA classification criteria using data from the ReACCh-Out (Research in Arthritis in Canadian Children, Emphasizing Outcomes) cohort. METHODS: We used clinicobiologic data recorded within 7 months of diagnosis to assign a diagnosis of JIA and identify subcategories of JIA among 1,228 patients according to the 2 JIA classification systems. We compared the proportions of patients classified and the alignment of classification categories with clinicobiologic subtypes and adult arthritis types. RESULTS: The PRINTO criteria classified 244 patients (19.9%) as having early-onset antinuclear antibody-positive JIA, 157 (12.8%) as having enthesitis/spondylitis-related JIA, 38 (3.1%) as having systemic JIA, and 10 (0.8%) as having rheumatoid factor-positive JIA. A total of 12% of patients were unclassifiable using the ILAR criteria, while 63.3% were unclassifiable using the PRINTO criteria (777 with other JIA and 2 with unclassified JIA). In sensitivity analyses, >50% of patients remained unclassifiable using the PRINTO criteria. Compared to the PRINTO criteria, ILAR JIA categories aligned better with clinicobiologic subtypes in 131 patients (χ2 = 44, P = 0.005, versus χ2 = 15, P = 0.07 for PRINTO), and ILAR categories aligned better with adult types of arthritis in 389 evaluable patients. CONCLUSION: Currently identified PRINTO disorders can only be used to classify a minority of JIA patients, leaving a large proportion of JIA patients with other disorders requiring further characterization. Current PRINTO JIA classification criteria do not align better with clinicobiologic subtypes or adult forms of arthritis compared with the older ILAR classification system.

Results From the 2020 Canadian Rheumatology Association's Workforce and Wellness Survey.

OBJECTIVE: The Canadian Rheumatology Association (CRA) launched the Workforce and Wellness Survey to update the Canadian rheumatology workforce characteristics. METHODS: The survey included demographic and practice information, pandemic effects, and the Mini Z survey to assess burnout. French and English survey versions were distributed to CRA members electronically between October 14, 2020, and March 5, 2021. The number of full-time equivalent (FTE) rheumatologists per 75,000 population was estimated from the median proportion of time in clinical practice multiplied by provincial rheumatologist numbers from the Canadian Medical Association. RESULTS: Forty-four percent (183/417) of the estimated practicing rheumatologists (149 adult; 34 pediatric) completed the survey. The median age was 47 years, 62% were female, and 28% planned to retire within the next 5-10 years. Respondents spent a median of 65% of their time in clinical practice. FTE rheumatologists per 75,000 population were 0.62 nationally and ranged between 0.00 and 0.70 in each province/territory. This represents a deficit of 1-78 FTE rheumatologists per province/territory and 194 FTE rheumatologists nationally to meet the CRA's workforce benchmark. Approximately half of survey respondents reported burnout (51%). Women were more likely to report burnout (OR 2.86, 95% CI 1.42-5.93). Older age was protective against burnout (OR 0.95, 95% CI 0.92-0.99). As a result of the pandemic, 97% of rheumatologists reported spending more time engaged in virtual care. CONCLUSION: There is a shortage of rheumatologists in Canada. This shortage may be compounded by the threat of burnout to workforce retention and productivity. Strategies to address these workforce issues are needed urgently.

Employment outcomes in systemic sclerosis.

Work disability is highly prevalent in the systemic sclerosis (SSc) population; yet, it is an area of research that continues to be underrecognized and underexplored. In this chapter, we review the burden of this work disability by exploring the reported prevalence of work loss, the risk factors associated with reduced work participation, the impact on work productivity outcomes, and the economic consequences of work disability in individuals with SSc. Finally, we discuss the potential challenges in the workplace and strategies that may foster employment retention in this population. We subsequently present a conceptual framework for work disability in the context of SSc, which incorporates our understanding of the various work disability concepts and the potential facilitators that may accelerate a worker toward complete work loss.

Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a complex group of systemic vasculitides that are characterized by primary small-to-medium sized blood vessel inflammation with the presence of autoantibodies known as ANCA. AAV diseases include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), and Microscopic Polyangiitis (MPA). AAVs are challenging conditions associated with high cumulative disease and treatment related morbidity and mortality. Given its rarity and the resulting paucity of pediatric-specific clinical trial evidence, pediatric rheumatologists have had to often extrapolate from adult literature for management and therapeutic decisions. The aim of this review is to provide a comprehensive overview of the important findings and overall conclusions of critical landmark clinical trials in the induction and maintenance treatments in adult AAV for the pediatric rheumatologist. This review also highlights the outcomes of recent pediatric AAV observational studies and discusses the future research priorities in pediatric AAV management.

Prospective Determination of the Incidence and Risk Factors of New-Onset Uveitis in Juvenile Idiopathic Arthritis: The Research in Arthritis in Canadian Children Emphasizing Outcomes Cohort.

OBJECTIVE: Identification of the incidence of juvenile idiopathic arthritis (JIA)-associated uveitis and its risk factors is essential to optimize early detection. Data from the Research in Arthritis in Canadian Children Emphasizing Outcomes inception cohort were used to estimate the annual incidence of new-onset uveitis following JIA diagnosis and to identify associated risk factors. METHODS: Data were reported every 6 months for 2 years, then yearly to 5 years. Incidence was determined by Kaplan-Meier estimators with time of JIA diagnosis as the reference point. Univariate log-rank analysis identified risk factors and Cox regression determined independent predictors. RESULTS: In total, 1,183 patients who enrolled within 6 months of JIA diagnosis met inclusion criteria, median age at diagnosis of 9.0 years (interquartile range [IQR] 3.8-12.9), median follow-up of 35.2 months (IQR 22.7-48.3). Of these patients, 87 developed uveitis after enrollment. The incidence of new-onset uveitis was 2.8% per year (95% confidence interval [95% CI] 2.0-3.5) in the first 5 years. The annual incidence decreased during follow-up but remained at 2.1% (95% CI 0-4.5) in the fifth year, although confidence intervals overlapped. Uveitis was associated with young age (<7 years) at JIA diagnosis (hazard ratio [HR] 8.29, P < 0.001), positive antinuclear antibody (ANA) test (HR 3.20, P < 0.001), oligoarthritis (HR 2.45, P = 0.002), polyarthritis rheumatoid factor negative (HR 1.65, P = 0.002), and female sex (HR 1.80, P = 0.02). In multivariable analysis, only young age at JIA diagnosis and ANA positivity were independent predictors of uveitis. CONCLUSION: Vigilant uveitis screening should continue for at least 5 years after JIA diagnosis, and priority for screening should be placed on young age (<7 years) at JIA diagnosis and a positive ANA test.

Systemic Juvenile Idiopathic Arthritis.

Systemic juvenile idiopathic arthritis (sJIA) is a distinctive subtype of juvenile idiopathic arthritis, characterized by fever and arthritis, often accompanied by rash, sometimes by generalized lymphadenopathy, hepatosplenomegaly, and serositis. The diagnosis requires adequate exclusion of infectious, oncologic, autoimmune, and autoinflammatory diseases. Macrophage activation syndrome, a serious and potentially fatal complication of sJIA, requires prompt evaluation and treatment. Newer biologic agents, particularly interleukin-1 and interleukin-6 inhibitors, are highly effective and have transformed the treatment approach by reducing the use of systemic glucocorticoids. Primary care providers have a crucial role in monitoring children with sJIA for disease-related complications and medication-related adverse events.

Health-Related Quality of Life in an Inception Cohort of Children With Juvenile Idiopathic Arthritis: A Longitudinal Analysis.

OBJECTIVE: To describe changes in health-related quality of life (HRQoL) over time in children with juvenile idiopathic arthritis (JIA), relative to other outcomes, and to identify predictors of unfavorable HRQoL trajectories. METHODS: Children with JIA in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort were included. The Juvenile Arthritis Quality of Life Questionnaire (JAQQ, a standardized instrument), health-related Quality of My Life (HRQoML, an instrument based on personal valuations), and JIA core variables were completed serially. Analyses included median values, Kaplan-Meier survival curves, and latent trajectory analysis. RESULTS: A total of 1,249 patients enrolled at a median of 0.5 months after diagnosis were followed for a median of 34.2 months. The degree of initial HRQoL impairment and probabilities of reaching the best possible HRQoL scores varied across JIA categories (best for oligoarthritis, worst for rheumatoid factor-positive polyarthritis). Median times to attain best possible HRQoL scores (JAQQ 59.3 months, HRQoML 34.5 months), lagged behind those for disease activity, pain, and disability measures. Most patients followed trajectories with minimal or mild impairment; however, 7.6% and 13.8% of patients, respectively, followed JAQQ and HRQoML trajectories with persistent major impairment in HRQoL. JIA category, aboriginal ethnicity, and baseline disease activity measures distinguished between membership in trajectories with major and minimal impairments. CONCLUSION: Improvement in HRQoL is slower than for disease activity, pain, and disability. Improvement of a measure based on respondents' preferences (HRQoML) is more rapid than that of a standardized measure (JAQQ). Higher disease activity at diagnosis heralds an unfavorable HRQoL trajectory.