Childhood HAQ Research Articles

Successful integration of an automated patient-reported outcome measure within a hospital electronic patient record.

ObjectivesThe objective of this evaluation was to assess the feasibility of implementing a fully integrated, automated, electronic patient-reported outcome measures (ePROM) system into a hospital electronic patient record (EPR; hospital-based clinical record). Additional objectives included evaluating the effect of the system on patient-reported outcome measures (PROM) completion rates and investigating the acceptability of the ePROM.MethodsThe evaluation was conducted in a rheumatology clinic in a specialist children’s hospital in the UK. Paper-based childhood HAQ PROMs were already used in the clinic, and an EPR was the main hospital information system. The technical feasibility of introducing the ePROM technology was assessed using a case study approach; the effect of the system on PROM completion rates was investigated using a before–after design; and acceptability was assessed using semi-structured questionnaires and a focus group.ResultsAn automated and integrated ePROM system was implemented successfully in April 2021. After implementation, ∼500 automated SMS text messaging invitations to complete ePROMs were sent to care-givers each month. PROM completion rates increased from 33 of 100 (33%) to 47 of 65 (72%) after the introduction of the ePROM system (χ2 = 11.51; P < 0.05). The ePROM system was highly acceptable to patients and clinical staff. Some clinical staff expressed a concern that an electronic system might represent a barrier to care for families with more limited resources.ConclusionHigh levels of automation and integration with existing technology systems seemed to be key contextual factors associated with the successful implementation and adoption of the ePROM intervention in a paediatric rheumatology clinic.

No evidence that genetic predictors of susceptibility predict changes in core outcomes in JIA.

ObjectivesThe clinical progression of JIA is unpredictable. Knowing who will develop severe disease could facilitate rapid intensification of therapies. We use genetic variants conferring susceptibility to JIA to predict disease outcome measures.MethodsA total of 713 JIA patients with genotype data and core outcome variables (COVs) at diagnosis (baseline) and 1 year follow-up were identified from the Childhood Arthritis Prospective Study (CAPS). A weighted genetic risk score (GRS) was generated, including all single nucleotide polymorphisms (SNPs) previously associated with JIA susceptibility (P-value < 5×10−08). We used multivariable linear regression to test the GRS for association with COVS (limited joint count, active joint count, physician global assessment, parent/patient general evaluation, childhood HAQ and ESR) at baseline and change in COVS from baseline to 1 year, adjusting for baseline COV and International League of Associations of Rheumatology (ILAR) category. The GRS was split into quintiles to identify high (quintile 5) and low (quintile 1) risk groups.ResultsPatients in the high-risk group for the GRS had a younger age at presentation (median low risk 7.79, median high risk 3.51). No association was observed between the GRS and any outcome measures at 1 year follow-up or baseline.ConclusionFor the first time we have used all known JIA genetic susceptibility loci (P=<5×10−08) in a GRS to predict changes in disease outcome measured over time. Genetic susceptibility variants are poor predictors of changes in core outcome measures, it is likely that genetic factors predicting disease outcome are independent to those predicting susceptibility. The next step will be to conduct a genome-wide association analysis of JIA outcome.

Prospective validation of the Juvenile Spondyloarthritis Disease Activity Index in children with enthesitis-related arthritis

To measure disease activity in children with enthesitis-related arthritis the Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was developed and retrospectively validated. We prospectively validated JSpADA and also assessed performance of adult SpA scores. Children with enthesitis-related arthritis (ILAR criteria) less than 18 years of age were enrolled. Baseline characteristics and different disease activity measures (JSpADA, BASDAI, Ankylosing Spondylitis Disease Activity Score-ESR, juvenile arthritis DAS-10 joints), and Childhood HAQ, physician global assessment and patient global assessment were recorded at baseline. In some children follow-up was also done. The mean (s.d.) age of 127 children (116 boys) was 14.3 (2.4) years and disease duration was 36.9 (3) months. Ninety of 104 (86.5%) children were HLA-B27 positive. JSpADA showed high correlation with physician global assessment (r = 0.87; P < 0.0001), patient global assessment (r = 0.80, P < 0.0001), juvenile arthritis DAS-10 joints (r = 0.89; P < 0.0001) and Childhood HAQ (r = 0.83, P < 0.0001). The JSpADA scores showed good internal consistency, discriminative validity and sensitivity to change. In 15% of children back mobility could not be tested due to active arthritis in lower limbs. The 7-variable JSpADA excluding back mobility performed as well as the original JSpADA. Adult scores showed good construct validity, discriminative capacity and sensitivity to change, and had good correlation with JSpADA (BASDAI, r = 0.84; Ankylosing Spondylitis Disease Activity Score-ESR, r = 0.84). JSpADA is a valid score for measuring disease activity in enthesitis-related arthritis. Adult scores also performed well. Excluding back mobility needs to be assessed in future to improve JSpADA performance.

Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes.

To report baseline articular, functional and ocular findings of the first international prospective cohort study of Blau syndrome (BS). Three-year, multicentre, observational study on articular, functional (HAQ, Childhood HAQ and VAS global and pain), ophthalmological, therapeutic and radiological data in BS patients. Baseline data on the first 31 recruited patients (12 females and 19 males) from 18 centres in 11 countries are presented. Of the 31 patients, 11 carried the p.R334W NOD2 mutation, 9 the p.R334Q and 11 various other NOD2 missense mutations; 20 patients were sporadic and 11 from five BS pedigrees. Median disease duration was 12.8 years (1.1-57). Arthritis, documented in all but one patient, was oligoarticular in 7, polyarticular in 23. The median active joint count was 21. Functional capacity was normal in 41%, mildly impaired in 31% and moderate-severe in 28% of patients. The most frequently involved joints at presentation were wrists, ankles, knees and PIPs. On radiographs, a symmetrical non-erosive arthropathy was shown. Previously unknown dysplastic bony changes were found in two-thirds of patients. Ocular disease was documented in 25 of 31 patients, with vitreous inflammation in 64% and moderate-severe visual loss in 33%. Expanded manifestations (visceral, vascular) beyond the classic clinical triad were seen in 52%. BS is associated with severe ocular and articular morbidity. Visceral involvement is common and may be life-threatening. Bone dysplastic changes may show diagnostic value and suggest a previously unknown role of NOD2 in bone morphogenesis. BS is resistant to current drugs, suggesting the need for novel targeted therapies.

Functional status in severe juvenile idiopathic arthritis in the biologic treatment era: an assessment in a French paediatric rheumatology referral centre

To investigate the functional status of difficult-to-treat JIA patients, including patients receiving biotherapies, and to correlate functional status to disease activity. All JIA patients consecutively evaluated in a paediatric rheumatology referral centre (November 2008 to March 2009) were enrolled in an observational cross-sectional study. The Childhood HAQ (CHAQ), physician's assessment of overall disease activity, parent's assessment of well-being and pain, and active and limited joint numbers were measured. We enrolled 95 patients [27% systemic, 29% polyarticular, 22% enthesitis-related arthritis (ERA) and 23% oligoarticular JIA]. Median disease duration was 3.5 years. Treatment included NSAIDs (56%), MTX (23%), CSs (21%) and biologics (45%). Of all patients, 31 and 56%, respectively, had inactive and minimally active disease. The median CHAQ score was 0.375 (range 0-3). Most patients had no or mild functional disability (61%), impaired well-being (63%) or pain (55%); 10% reported severely impaired function and well-being, 19% severe pain. ERA patients reported worse well-being and pain. CHAQ scores correlated with disease activity. Long-lasting disease and biologic treatment were associated with better well-being and pain scores. Despite the high proportion of severe JIA patients in this cohort, CHAQ values are within the lower range of recent reports, probably related to new therapeutic approaches. Impaired function and well-being remain a challenge for at least 10% of the patients. Impaired well-being and pain in ERA patients require further study. The strong correlation between functional status and well-being underlines the importance of improving function to optimize quality of life.

Complete control of disease activity and remission induced by treatment with etanercept in juvenile idiopathic arthritis

To identify contributing factors associated with inactive disease (ID) and clinical remission for patients with juvenile idiopathic arthritis (JIA) treated with etanercept. Data of an ongoing, long-term, multicentre, prospective, open-label observational study were analysed. Results. A total of 4898 follow-up forms from 787 JIA patients treated with etanercept were evaluated. Sixty-four per cent of the patients were female, 75% concomitantly received MTX and 45.9% received corticosteroids. Until the last observation, 47.6% of patients reached the criteria for ID and 26.6% achieved the remission on medication criteria. For both, ID and remission on medication, a significant influence of shorter disease duration (P<0.01 and P<0.001), a weekly dosage of at least 0.8 mg/kg (P=0.02), lower active joint counts (P=0.001) and lower childhood HAQ score (P<0.001 and P=0.004) at baseline was found. The odds ratio (OR) for a girl's chance to reach ID was 0.73 (P=0.049) and for remission on medication was 0.68 (P=0.04) compared with boys. The concomitant administration of MTX raised the relative chance, especially in patients with seronegative polyarthritis (OR 2.0; P=0.03). Upon treatment with etanercept, many JIA patients reached ID and remission on medication, although they had suffered from long-standing, refractory disease. Earlier initiation of treatment, less severe disease, use of at least the recommended weekly dosage of etanercept, and concomitant treatment with MTX seemed to independently increase the chance for reaching remission. These data should be confirmed by controlled prospective studies.

Disease activity and disability in children with juvenile idiopathic arthritis one year following presentation to paediatric rheumatology. Results from the Childhood Arthritis Prospective Study

Objective. Inflammatory arthritis in childhood is variable in terms of both presentation and outcome. This analysis describes disease activity in children with juvenile idiopathic arthritis (JIA) during the first year following presentation to a paediatric rheumatologist and identifies predictors of moderate to severe disability [defined using a Childhood HAQ (CHAQ) score ⩾0.75] at 1 year.Methods. The Childhood Arthritis Prospective Study recruits children <16 years with new inflammatory arthritis persisting for ⩾2 weeks from five UK tertiary referral centres. Demographics, disease features, joint count, CHAQ, physician's global assessment, parent's general evaluation of well-being (PGE), ESR and treatment, are collected at first presentation, 6 months and then yearly. Independent predictors of CHAQ ⩾0.75 at 1 year in children diagnosed with JIA were identified using multivariable logistic regression models.Results. Seven hundred and forty children with JIA were included; median age at presentation 7.6 years, 64% girls. During the first year, 85% received NSAIDs, 70% IA corticosteroids, 47% MTX and 27% systemic steroids (oral or i.v.). Median presenting CHAQ score was 0.63 and decreased to 0.25 at 1 year; 32% had CHAQ ⩾0.75 at 1 year. The strongest predictor of CHAQ ⩾0.75 at 1 year was CHAQ ⩾0.75 at presentation (odds ratio 3.92; 95% CI 2.17, 7.09). Additional predictors included female gender and higher PGEConclusion. Although CHAQ score improved in most children, the strongest predictor of persistent disability at 1 year was moderate to severe disability at first presentation. Follow-up beyond 1 year will assess whether CHAQ at presentation will continue to be a predictor of future poor outcome.

Dynamic contrast-enhanced magnetic resonance imaging in the assessment of disease activity in patients with juvenile idiopathic arthritis

To determine the capability and reliability of dynamic contrast-enhanced MRI (DCE-MRI) in the assessment of disease activity in juvenile idiopathic arthritis (JIA). DCE-MRI of the clinically more affected wrist or hip joints was undertaken in 21 patients, coupled with standard clinical assessment and biochemical analysis. Synovial inflammation was assessed by computing the maximum level of synovial enhancement (ME), the maximum rate of enhancement (MV) and the rate of early enhancement (REE) from the enhancement curves generated from region of interest independently delineated by two readers in the area of the ME. Correlations between dynamic parameters and clinical measures of disease activity, and static MRI synovitis score were investigated. In patients with wrist arthritis, REE correlated with the wrist swelling score (r(s) = 0.72), ESR (r(s) = 0.69), pain assessment scale (r(s) = 0.63) and childhood HAQ (r(s) = 0.60). In patients with hip arthritis, ME correlated with the hip limitation of motion (r(s) = 0.69). Static MRI synovitis score based on post-gadolinium enhancement correlated with MV (r(s) = 0.63) in patients with wrist arthritis and with ME (r = 0.68) in those with hip arthritis. The inter-reader agreement assessed by intra-class correlation coefficient (ICC) for ME, MV and REE (ICC = 0.98, 0.97 and 0.84, respectively) was excellent. DCE-MRI represents a promising method for the assessment of disease activity in JIA, especially in patients with wrist arthritis. As far as we know, this study is the first to demonstrate the feasibility, reliability and construct validity of DCE-MRI in JIA. These results should be confirmed in large-scale longitudinal studies in view of its further application in therapeutic decision making and in clinical trials.

Development of a digital Childhood Health Assessment Questionnaire for systematic monitoring of disease activity in daily practice

To develop a reliable and user-friendly digital Childhood HAQ (CHAQ) to facilitate systematic monitoring of disease activity at the outpatient clinic in juvenile idiopathic arthritis (JIA) patients. The digital CHAQ was tested with patients who visited the outpatient paediatric rheumatology clinic of the Erasmus MC Sophia Children's Hospital. These patients completed in a randomized order the paper form and digital CHAQ while being observed. Validity was tested by comparing outcomes with the paper form CHAQ. User-friendliness was evaluated through a short questionnaire. A digital CHAQ was developed and revised several times according to our observations. Outcome is automatically calculated and can be printed. Fifty-one patients completed both the digital and paper form CHAQ. Correlation coefficient between both outcomes of the CHAQ Disability Index was 0.974. No statistically significantly differences in median outcome were found in visual analogue scale (VAS) pain (25.6 vs 25.9 mm) and VAS well-being (20.1 vs 19.5 mm). Although the mean time (5.06 min) to complete the digital CHAQ was significantly longer than the mean time (3.75 min) to complete the paper form, the majority of patients (75%) preferred the digital version. User-friendliness received maximum positive score. We developed a reliable and user-friendly digital CHAQ, which can be easily and systematically completed during routine clinic visits. Such digitalization of questionnaires can be applied in any field to make systematic monitoring of disease activity in daily practice possible.

Psychological functioning of children and adolescents with juvenile idiopathic arthritis is related to physical disability but not to disease status

This study investigates the psychological functioning of children with polyarticular joint disease and its association with disease activity and disability. Sixty children aged 7-18 yrs with juvenile idiopathic arthritis and >4 joints involved were recruited. Children underwent a physical examination. The Childhood HAQ was completed by both the children and their parents. Children also completed questionnaires for depression (Birleson Depression Inventory; BDI), anxiety (Revised Children's Manifest Anxiety Scale; RCMAS) and peer, emotional and behavioural problems (Strengths and Difficulties Questionnaire; SDQ). Clinical information was extracted from the hospital records. Self-reported psychological functioning (depression, anxiety, and behaviour) was not different from the normal population. Parent-reported emotional difficulties on the SDQ were somewhat elevated. There were no significant correlations between psychological functioning and physician-rated disease activity score or the number of active joints at the time of assessment. Furthermore, no differences in psychological functioning were found between children with or without significantly raised inflammatory markers. All aspects of psychological function (depression, anxiety and behaviour) correlated moderately with physical function (r(s) = 0.49, 0.41, 0.46, respectively; all P < 0.01). Children and adolescents with polyarthritis are not at significantly elevated risk of psychological difficulties. Poor psychological outcome was associated with more severe physical disability but not with the level of disease activity.

P17 Juvenile arthritis damage index (JADI) has a good correlation with childhood HAQ and radiological abnormalities in juvenile idiopathic arthritis

P17 Juvenile arthritis damage index (JADI) has a good correlation with childhood HAQ and radiological abnormalities in juvenile idiopathic arthritis