Search

The goal was to assess the degree of overlap between existing International League of Associations for Rheumatology (ILAR) and preliminary Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria for juvenile idiopathic arthritis (JIA). Participants from the Childhood Arthritis Prospective Study, a multicenter UK JIA inception cohort, were classified using the PRINTO and ILAR classification criteria into distinct categories. Systemic JIA was excluded because several classification items were not collected in this cohort. Adaptations to PRINTO criteria were required to apply to a UK health care setting, including limiting the number of blood biomarker tests required. The overlap between categories under the two systems was determined, and any differences in characteristics between groups were described. A total of 1,223 children and young people with a physician’s diagnosis of JIA were included. Using PRINTO criteria, the majority of the patients had “other JIA” (69.5%). There was a high degree of overlap (91%) between the PRINTO enthesitis/spondylitis‐ and ILAR enthesitis‐related JIA categories. The PRINTO rheumatoid factor (RF)–positive category was composed of 48% ILAR RF‐positive polyarthritis and 52% undifferentiated JIA. The early‐onset antinuclear antibodies–positive PRINTO category was largely composed of ILAR oligoarthritis (50%), RF‐negative polyarthritis (24%), and undifferentiated JIA (23%). A few patients were unclassified under PRINTO (n = 3) and would previously have been classified as enthesitis‐related JIA (n = 1) and undifferentiated JIA (n = 2) under ILAR. Under the preliminary PRINTO classification criteria for childhood arthritis, most children are not yet classified into a named category. These data can help support further delineation of the PRINTO criteria to ensure homogenous groups of children can be identified.

ObjectiveTo investigate the relationship between age and symptom duration at initial presentation to pediatric rheumatology for juvenile idiopathic arthritis (JIA).MethodsIn children and young people (CYP) enrolled in the Childhood Arthritis Prospective Study prior to March 2018, an association between age at presentation (< 5, 5–11, and > 11 yrs) and symptom duration was tested by multivariable linear regression.ResultsIn 1577 CYP, 5- to 11-year-olds took 3.2 months longer and > 11-year-olds 6.9 months longer to reach pediatric rheumatology than < 5-year-olds.ConclusionAdolescents take longer to reach pediatric rheumatology, potentially affecting their longer-term outcomes given the window of opportunity for JIA treatment.

As young people enter adulthood, the interchangeable use of child and adult outcome measures may inaccurately capture changes over time. This study aimed to use item response theory (IRT) to model a continuous score for functional ability that can be used no matter which questionnaire is completed. Adolescents (ages 11–17 years) in the UK Childhood Arthritis Prospective Study (CAPS) self‐completed an adolescent Childhood Health Assessment Questionnaire (CHAQ) and a Health Assessment Questionnaire (HAQ). Their parents answered the proxy‐completed CHAQ. Those children with at least 2 simultaneously completed questionnaires at initial presentation or 1 year were included. Psychometric properties of item responses within each questionnaire were tested using Mokken analyses to assess the applicability of IRT modeling. A previously developed IRT model from the Pharmachild‐NL registry from The Netherlands was validated in CAPS participants. Agreement and correlations between IRT‐scaled functional ability scores were tested using intraclass correlations and Wilcoxon’s signed rank tests. In 303 adolescents, the median age at diagnosis was 13 years, and 61% were female. CHAQ scores consistently exceeded HAQ scores. Mokken analyses demonstrated high scalability, monotonicity, and the fact that each questionnaire yielded reliable scores. There was little difference in item response characteristics between adolescents enrolled in CAPS and Pharmachild‐NL (maximum item residual 0.08). Significant differences were no longer evident between IRT‐scaled HAQ and CHAQ scores. IRT modeling allows the direct comparison of function scores regardless of different questionnaires being completed by different people over time. IRT modeling facilitates the ongoing assessment of function as adolescents transfer from pediatric clinics to adult services.

In pediatric research, investigators rely on proxy reports of outcome, such as the proxy‐completed Childhood Health Assessment Questionnaire (C‐HAQ), to assess function in juvenile idiopathic arthritis (JIA). As children mature, they may self‐complete the adult HAQor the unvalidated adolescent‐specific C‐HAQ. It is unclear how these measures compare and whether they are directly interchangeable. The present study was undertaken to compare agreement between the proxy‐completed C‐HAQ, adolescent‐specific C‐HAQ, and the HAQ at initial presentation to pediatric rheumatologic care and 1 year following the first presentation in adolescents with JIA. Adolescents ages 11–17 years participating in the Childhood Arthritis Prospective Study (CAPS), a UKmulticenter inception cohort, were included. In a CAPSsubstudy, adolescents self‐completed the adolescent‐specific C‐HAQand the HAQ, and proxies simultaneously completed the proxy‐completed C‐HAQat baseline and 1 year. Correlation and agreement between scores were assessed at baseline. Agreement and ability to similarly classify clinically important changes over time were assessed at 1 year following initial presentation to rheumatologic care. A total of 107 adolescents (adolescent‐specific C‐HAQand HAQ) or their proxies (proxy‐completed C‐HAQ) had completed all 3 measures at baseline. Median age at diagnosis was 13 years, and 61% were female. Although the 3 scores demonstrated strong correlations (r > 0.8), they were not completely interchangeable, with agreement ranging between 70% and 80%. There was similar agreement between the changes in scores between baseline and 1 year. Using proxy‐completed C‐HAQminimum clinically important cutoffs, the adolescent‐specific C‐HAQand the HAQsimilarly classified 80% to 90% of adolescents as having improved or worsened. While there is relatively high agreement and similar classification of change between HAQand the 2 C‐HAQscores, these are not completely interchangeable. This impacts the comparison of function when measured in different ways over the lifespan.

The aims of treating juvenile idiopathic arthritis are to elicit treatment response toward remission, while preventing future flares. Understanding patient and disease characteristics that predispose young people with this condition to these outcomes would allow the forecasting of disease process and the tailoring of therapies. The strongest predictor of remission is disease category, particularly oligoarthritis, although a few additional clinical predictors of treatment response have been identified. Novel evidence using biomarkers, such as S100 proteins and novel single nucleotide polymorphism data, could add value to clinical models. The future aim of personalised medicine in the treatment of juvenile idiopathic arthritis will be aided with international collaborations, allowing for the analysis of larger datasets with novel biomarker data. Combined clinical and biomarker panels will probably be required for predicting outcomes in such a complex disease.

Juvenile idiopathic arthritis (JIA) is the most common inflammatory joint condition of childhood and represents seven JIA subtypes characterised by distinct clinical and laboratory variables. Genetic and environmental factors are known to influence JIA, although many unanswered questions remain. Measurement of health outcomes in JIA is imperative for both clinical practice and research. Patient-reported outcomes present particular challenges in paediatric rheumatology in view of the importance of collecting reports from both the child/young person and the parent. Another challenge is the need for continuity of outcome measurement across the paediatric–adult interface during the process of transition in terms of both measurement tools and the mechanisms in the system to facilitate tracking of the young person into adult care. Finally, the need for adults with JIA to be seen as a distinct group in adult rheumatology practice is important for both service provision and outcome research.

Despite its diagnostic and prognostic importance, physiologic dead space fraction is not included in the current ARDS definition or severity classification. ARDS caused by COVID-19 (C-ARDS) is characterized by increased physiologic dead space fraction and hypoxemia. Our aim was to investigate the relationship between dead space indices, markers of inflammation, immunothrombosis, severity and intensive care unit (ICU) mortality.