Natalie J. Shiff

The outcomes of juvenile idiopathic arthritis in children managed with contemporary treatments: results from the ReACCh-Out cohort

Objective To describe clinical outcomes of juvenile idiopathic arthritis (JIA) in a prospective inception cohort of children managed with contemporary treatments. Methods Children newly diagnosed with JIA at 16 Canadian paediatric rheumatology centres from 2005 to 2010 were included. Kaplan–Meier survival curves for each JIA category were used to estimate probability of ever attaining an active joint count of 0, inactive disease (no active joints, no extraarticular manifestations and a physician global assessment of disease activity <10 mm), disease remission (inactive disease >12 months after discontinuing treatment) and of receiving specific treatments. Results In a cohort of 1104 children, the probabilities of attaining an active joint count of 0 exceeded 78% within 2 years in all JIA categories. The probability of attaining inactive disease exceeded 70% within 2 years in all categories, except for RF-positive polyarthritis (48%). The probability of discontinuing treatment at least once was 67% within 5 years. The probability of attaining remission within 5 years was 46–57% across JIA categories except for polyarthritis (0% RF-positive, 14% RF-negative). Initial treatment included joint injections and non-steroidal anti-inflammatory drugs for oligoarthritis, disease-modifying antirheumatic drugs (DMARDs) for polyarthritis and systemic corticosteroids for systemic JIA. Conclusions Most children with JIA managed with contemporary treatments attain inactive disease within 2 years of diagnosis and many are able to discontinue treatment. The probability of attaining remission within 5 years of diagnosis is about 50%, except for children with polyarthritis.

The risk and nature of flares in juvenile idiopathic arthritis: results from the ReACCh-Out cohort

Objective To describe probabilities and characteristics of disease flares in children with juvenile idiopathic arthritis (JIA) and to identify clinical features associated with an increased risk of flare. Methods We studied children in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) prospective inception cohort. A flare was defined as a recurrence of disease manifestations after attaining inactive disease and was called significant if it required intensification of treatment. Probability of first flare was calculated with Kaplan–Meier methods, and associated features were identified using Cox regression. Results 1146 children were followed up a median of 24 months after attaining inactive disease. We observed 627 first flares (54.7% of patients) with median active joint count of 1, physician global assessment (PGA) of 12 mm and duration of 27 weeks. Within a year after attaining inactive disease, the probability of flare was 42.5% (95% CI 39% to 46%) for any flare and 26.6% (24% to 30%) for a significant flare. Within a year after stopping treatment, it was 31.7% (28% to 36%) and 25.0% (21% to 29%), respectively. A maximum PGA >30 mm, maximum active joint count >4, rheumatoid factor (RF)-positive polyarthritis, antinuclear antibodies (ANA) and receiving disease-modifying antirheumatic drugs (DMARDs) or biological agents before attaining inactive disease were associated with increased risk of flare. Systemic JIA was associated with the lowest risk of flare. Conclusions In this real-practice JIA cohort, flares were frequent, usually involved a few swollen joints for an average of 6 months and 60% led to treatment intensification. Children with a severe disease course had an increased risk of flare.

Unpacking Early Work Experiences of Young Adults With Rheumatic Disease: An Examination of Absenteeism, Job Disruptions, and Productivity Loss

To examine work absenteeism, job disruptions, and perceived productivity loss and factors associated with each outcome in young adults living with systemic lupus erythematosus (SLE) and juvenile arthritis (JA).

What Matters Most for Patients, Parents, and Clinicians in the Course of Juvenile Idiopathic Arthritis? A Qualitative Study

Objective. To assess which clinical features are most important for patients, parents, and clinicians in the course of juvenile idiopathic arthritis (JIA). Methods. Forty-nine people participated in 6 audience-specific focus group discussions and 112 reciprocal interviews in 3 Canadian cities. Participants included youth with JIA, experienced English- and French-speaking parents, novice parents (< 6 mos since diagnosis), pediatric rheumatologists, and allied health professionals. Participants discussed the importance of 34 JIA clinical features extracted from medical literature. Transcripts and interview reports underwent qualitative analysis to establish relative priorities for each group. Results. Most study participants considered medication requirements, medication side effects, pain, participant-defined quality of life, and active joints as high priority clinical features of JIA. Active joint count was the only American College of Rheumatology core variable accorded high or medium priority by all groups. Rheumatologists and allied health professionals considered physician global assessment as high priority, but it had very low priority for patients and parents. The parent global assessment was considered high priority by clinicians, medium to high by parents, and low by patients. Child Health Assessment Questionnaire scores were considered low priority by patients and parents, and moderate or high by clinicians. The number of joints with limited motion was given low to very low priority by all groups. Parents gave high priority to arthritis flares. Conclusion. If our findings are confirmed, medication requirements, medication side effects, pain, participant-defined quality of life, and active joint counts should figure prominently in describing the course of JIA.

Access to Pediatric Rheumatology Subspecialty Care in British Columbia, Canada

Objective. Early recognition and treatment of pediatric rheumatic diseases is associated with improved outcome. We documented access to pediatric rheumatology subspecialty care for children in British Columbia (BC), Canada, referred to the pediatric rheumatology clinic at BC Children’s Hospital, Vancouver. Methods. An audit of new patients attending the outpatient clinic from May 2006 to February 2007 was conducted. Parents completed a questionnaire through a guided interview at the initial clinic assessment. Referral dates were obtained from the referral letters. Patients were classified as having rheumatic disease, nonrheumatic disease, or a pain syndrome based on final diagnosis by a pediatric rheumatologist. Results. Data were collected from 124 of 203 eligible new patients. Before pediatric rheumatology assessment, a median of 3 healthcare providers were seen (range 1–11) for a median of 5 visits (range 1–39). Overall, the median time interval from symptom onset to pediatric rheumatology assessment was 268 days (range 13–4989), and the median time interval from symptom onset to referral to pediatric rheumatology was 179 days (range 3–4970). Among patients ultimately diagnosed with rheumatic diseases (n = 53), there was a median of 119 days (range 3–4970) from symptom onset to referral, and 169 days (range 31–4989) from onset to pediatric rheumatology assessment. Conclusion. Children and adolescents with rheumatic complaints see multiple care providers for multiple visits before referral to pediatric rheumatology, and there is often a long interval between symptom onset and this referral.

Validation of administrative health data for the pediatric population: a scoping review.

BackgroundThe purpose of this research was to perform a scoping review of published literature on the validity of administrative health data for ascertaining health conditions in the pediatric population (≤20 years).MethodsA comprehensive search of OVID Medline (1946 - present), CINAHL (1937 - present) and EMBASE (1947 - present) was conducted. Characteristics of validation studies that were abstracted included the study population, health condition, topic of the validation (e.g., single diagnosis code versus case-finding algorithm), administrative and validation data sources. Inter-rater agreement was measured using Cohen’s κ. Extracted data were analyzed using descriptive statistics.ResultsA total of 37 articles met the study inclusion criteria. Cohen’s κ for study inclusion/exclusion and data abstraction was 0.88 and 0.97, respectively. Most studies validated administrative data from the USA (43.2%) and Canada (24.3%), and focused on inpatient records (67.6%). Case-finding algorithms (56.7%) were more frequently validated than diagnoses codes alone (37.8%). Five conditions were validated in more than one study: diabetes mellitus, inflammatory bowel disease, asthma, rotavirus infection, and tuberculosis.ConclusionsThis scoping review identified a number of gaps in the validation of administrative health data for pediatric populations, including limited investigation of outpatient populations and older pediatric age groups.

Glucocorticoid-Related Changes in Body Mass Index Among Children and Adolescents With Rheumatic Diseases

To examine the temporal and dose‐related effects of glucocorticoids (GCs) on body mass index (BMI) in children with rheumatic diseases.

Factors Associated with a Longer Time to Access Pediatric Rheumatologists in Canadian Children with Juvenile Idiopathic Arthritis

Objective. The Research on Arthritis in Canadian Children Emphasizing Outcomes (ReACCh Out) cohort is a prospective inception cohort of patients with newly diagnosed juvenile idiopathic arthritis (JIA) seen in 16 Canadian pediatric rheumatology (PR) centers. We used data from this cohort to explore factors associated with longer time from symptom onset to the first visit to (PR), and with longer time from first visit to a diagnosis of JIA. Methods. We included children enrolled in ReACCh Out within 6 months of JIA diagnosis, for whom the dates of symptom onset and first PR visit were recorded. We used Cox proportional hazard modeling to investigate the effects of history, physical examination, and laboratory evaluation on the interval from JIA symptom onset to first PR assessment. Results. In total, 319 children from the cohort were included. Having a fever (hazard ratio 1.80, 95% CI 1.10, 2.93), any part South Asian ethnicity (HR 1.75, 95% CI 1.04, 2.95), highly educated parents (HR 1.69, 95% CI 1.18, 2.44), and limp (HR 1.55, 95% 1.16, 2.06) were significantly associated with shorter time from symptom onset to first PR assessment, while a history of heel pain or enthesitis (HR 0.61, 95% 0.38, 0.97) was significantly associated with a longer time to first PR visit. Conclusion. Children with a history of a fever, limp, any part South Asian ethnicity, or highly educated parents were more likely to see PR sooner than patients without these features, while children with a history of enthesitis received PR care later than those without enthesitis.

Spectroscopic assessment of dermal melanin using blue vitiligo as an in vivo model

Background: Spectroscopic methods have been used to analyze in vivo melanin in the past but the specific effect of melanin depth on autofluorescence and reflectance spectroscopy has not been determined. In patients with blue vitiligo, three distinctive clinicopathologic patterns are present: (1) normal skin with normal epidermal melanin pigmentation (2), skin of blue vitiligo with dermal melanin pigmentation, and (3) tissue of regular vitiligo with no melanin pigmentation. Blue vitiligo may thus serve as an in vivo model to assess dermal pigment using spectroscopic techniques.

Transitioning to Employment with a Rheumatic Disease: The Role of Independence, Overprotection, and Social Support

Objective. To examine perceived independence, overprotection, and support, and their association with the employment participation of young adults with rheumatic disease. Methods. One hundred and forty-three young adults, ages 18 to 30 years, with systemic lupus erythematosus (54.5%) and juvenile arthritis (45.5%) completed a 30-min online questionnaire of their work and education experiences. Information collected was demographic, health (e.g., pain, fatigue, disease activity), work context (e.g., career satisfaction, helpfulness of job accommodation/benefits, and workplace activity limitations), and psychosocial (e.g., independence, social support, and overprotection). Log-Poisson regression analysis examined factors associated with employment status. Results. Over half of respondents were employed (59%) and 26% were enrolled in school. Respondents reported moderate to high perceptions of independence and social support. However, 27% reported that “quite a bit” to “a great deal” of overprotection characterized their relationships with those closest to them. At the bivariate level, employed participants and those indicating greater perceived independence reported greater social support and less overprotection. Multivariable analysis revealed that being employed was associated with older age, more job accommodations/benefits perceived as being helpful, and greater perceived independence. Conclusion. This is one of the first studies examining the employment of young adults with rheumatic diseases. Findings highlight the importance of psychosocial perceptions such as independence and overprotection, in addition to support related to working. Additional research is needed to better understand the role of those close to young adults with rheumatic diseases in supporting independence and encouraging employment.