Childhood Health Assessment Questionnaire Disability Index Research Articles

Protocols in pediatric rheumatology (PROKIND): treat-to-target in polyarticular juvenile idiopathic arthritis

The aims of the PROKIND protocols are improvement and harmonization of the diagnostics, monitoring, treatment decision and prognosis. This article reports the results of a prospective treat-to-target observational study of patients with polyarticular juvenile idiopathic arthritis (JIA) during the first year of treatment. Disease activity was assessed with the 10-joint juvenile arthritis disease activity score (JADAS-10), functional limitation with the childhood health assessment questionnaire disability index (CHAQ-DI) and with information on overall well-being, on pain, on fatigue and on global estimation of disease activity. Overall, 129 patients with polyarticular JIA (rheumatoid factor, RF, positive (+) polyarthritis n = 22, RF negative (-) polyarthritis n = 133 from 23pediatric rheumatology institutions in Germany and Austria were recruited. Patients with initial treatment with methotrexate formed cohort1, patients with additional repeated intravenous corticosteroid pulse therapy formed cohort2 and patients with concomitant intra-articular corticosteroid administration in at least 5joints formed cohort3. The mean JADAS10 showed adecrease in disease activity from 16.4 ± 6.1 to 2.8 ± 3.6 and the decrease in the CHAQ-DI from 1.0 ± 0.8 to 0.3 ± 0.5 showed the improvement in functional capacity. Similarly, improvements in quality of life, pain and fatigue were demonstrable. A JADAS inactive disease was achieved by 18.1% at month3, 47.7% at month6 and66.7% at month12. In cohort 1 a JADAS remission was achieved by 72.4%, by 50% in cohort 2 and by 69.2% in cohort 3. An escalation to treatment with biologics was necessary in 38% of patients in cohort 1, 60% in cohort 2 and 46% in cohort 3. Using atreat-to-target approach a dramatic improvement in disease activity, functional capacity and quality of life in polyarticular JIA could be achieved. Even after 12months an inactive disease was achieved in the majority of cases.

Patient-Reported Outcomes Among Patients Ages Two to Seventeen Years With Polyarticular-Course Juvenile Idiopathic Arthritis Treated With Subcutaneous Abatacept: Two-Year Results From an International Phase III Study.

To describe longitudinal changes in patient-reported outcomes (PROs) in children with polyarticular-course juvenile idiopathic arthritis (pJIA) treated with subcutaneous abatacept. Secondary analysis of a single-arm, open-label 24-month study of patients ages 6-17 years and 2-5 years. PROs included Childhood Health Assessment Questionnaire-Disability Index (CHAQ-DI), parent global assessment of child well-being (PaGA), pain assessment, and Activity Limitation Questionnaire (ALQ). Clinical outcomes included 50% or greater improvement in JIA American College of Rheumatology (ACR) criteria, clinically inactive disease, and Juvenile Arthritis Disease Activity Score. For the 6- to 17-year-old (n=173) and 2- to 5-year-old (n=46) cohorts, respectively, median (Q1, Q3) changes from baseline in CHAQ-DI at months 4 and 24 were -0.3 (-0.8, 0.0) and -0.5 (-1.0, -0.1), and -0.4 (-0.8, 0.0) and -0.5 (-1.0--0.1). Median pain scores were below cutoff threshold for clinically relevant pain (<35 mm) by month 1 (6 to 17 years, 32.3 mm; 2 to 5 years, 25.7 mm), reaching a nadir at month 24 (6 to 17 years, 6.0 mm; 2 to 5 years, 2.0 mm). For the 6- to 17-year-old and 2- to 5-year-old cohorts, respectively, median PaGA scores were 47.8 (n=172) and 42.1 (n=46) at baseline and 6.3 (n=107) and 2.0 (n=37) at month 24. In both cohorts, ALQ components improved from baseline to month 4 and were largely maintained to month 24. Clinical outcomes improved through to month 24. Early and sustained PRO improvements were reported in this phase III, open-label trial of subcutaneous abatacept in patients with pJIA.

Heritable Connective Tissue Disorders in Childhood: Increased Fatigue, Pain, Disability and Decreased General Health.

Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires. Methods. This observational, multicenter study included 107 children, aged 4–18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent–Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ). Results. Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, rs = 0.68), pain (p < 0.001, rs = 0.64) and general health (p < 0.001, rs = 0.59). Conclusions. Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care.

Utilizing ultrasound findings of a single indicator joint to assess non-systemic juvenile idiopathic arthritis

BackgroundMusculoskeletal ultrasound (MSUS) has been used worldwide in adult patients with rheumatoid arthritis (RA) but is beginning to play an increasing role in patients with juvenile idiopathic arthritis (JIA). The aim of this study was to investigate the application of MSUS findings of a single indicator joint in JIA to assess the disease activity and classify disease subtype.MethodsThirty-five non-systemic JIA patients with a total of 62 visits were retrospectively recruited in this study. Among the involved joints, the joint with highest value of grey-scale (GS) plus power Doppler (PD) (=GSPD) was selected as the indicator joint at each visit. The correlations between each MSUS parameter (GS, PD, GSPD) of indicator joints and the Physician Global Assessment (PGA) score, the Childhood Health Assessment Questionnaire-disability index (CHAQ-DI), and laboratory data were analyzed. The ultrasound features in different subtypes of JIA were also compared.ResultsPD was weakly correlated with the PGA score (rho = 0.323, p = 0.010), while both GS and GSPD were moderately correlated with the PGA score (rho = 0.405, p = 0.001; rho = 0.434, p = 0.000). On the other hand, GS, PD, and GSPD were weakly correlated with CHAQ-DI. Although erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) had a weak correlation with PGA, they were not statistically correlated with GS, PD, or GSPD. The proportions of effusion, synovial hypertrophy, and enthesopathy in three different subtypes, showed significant differences (Fisher’s exact test, p = 0.037; p = 0.004; p = 0.019). Enthesopathy was only seen in joints of enthesitis-related arthritis (ERA), but not in joints of polyarthritis and oligoarthritis.ConclusionsMSUS is an acceptable non-invasive tool for the patients with JIA, particularly for those with non-systemic JIA, that might assist disease classification, and whose parameters of the indicator joints may potentially contribute to the evaluation of disease activity.

Comparing the Measurement Properties and Preferability of Patient-reported Outcome Measures in Pediatric Rheumatology: PROMIS vs CHAQ.

The Childhood Health Assessment Questionnaire (CHAQ), though widely used for assessments in pediatric rheumatology, has drawbacks, including low correlation to disease activity and ceiling effects. We sought to determine if any tools from the Patient Reported Outcomes Measurement Information System (PROMIS) improve on these shortcomings and/or are preferred by patients. Patients 5-17 years of age with juvenile idiopathic arthritis (JIA) or juvenile dermatomyositis (JDM) were recruited from the rheumatology clinics at a Canadian children's hospital. Participants completed the CHAQ, 3 PROMIS measures (pain interference, mobility, and physical activity), and underwent a standard clinical assessment. Fifty-two patients participated, 25 with JIA and 27 with JDM. None of the PROMIS measures suffered from ceiling effects, whereas the CHAQ Disability Index (DI) and pain visual analog scales both did, with 50% and 20% of patients achieving the best possible scores, respectively. The PROMIS mobility was moderately correlated to the CHAQ-DI (rs -0.60, 95% CI -0.75 to -0.40), and the PROMIS pain interference was strongly correlated to the CHAQ pain score (rs 0.65, 95% CI 0.43-0.80). No measures correlated with disease activity. Patients preferred the PROMIS to the CHAQ. The PROMIS pain interference, mobility, and physical activity measures improve in some areas where the CHAQ is weak: they do not suffer from ceiling effects, and patients prefer the PROMIS tools. More work is needed to determine the correlation and responsiveness of the PROMIS tools to changes in disease activity over time before they should be widely adopted for clinical use.

Prevalence of Depression in Patients with Juvenile Idiopathic Arthritis Presenting at a Tertiary Care Hospital.

Background and objectiveJuvenile idiopathic arthritis (JIA) is an idiopathic autoimmune rheumatic disorder in children. JIA has been associated with depression and has a negative psychological impact on patients' quality of life. The aim of the study is to determine the prevalence of depression in patients with JIA presenting at a tertiary care hospital in Islamabad, Pakistan.Materials and methodsThis cross-sectional study, conducted at the Department of Rheumatology, Pakistan Institute of Medical Sciences, Islamabad, Pakistan, included 100 children aged >6 years who had been diagnosed with JIA according to the 2004 revised International League of Associations for Rheumatology classification. Physical disability was measured using the Childhood Health Assessment Questionnaire disability index (CHAQ-DI), whereas depression was assessed by measuring their Center for Epidemiological Studies Depression Scale for Children (CES-DC) scores. Results were analyzed using IBM SPSS Statistics for Windows, Version 20.0. (Armonk, NY: IBM Corp.), with p-values ≤ 0.05 considered statistically significant.ResultsThe 100 patients included 54 male patients (mean age, 16.3 ± 4.9 years) and 46 female patients (mean age, 18.6 ± 5.1 years). CES-DC scores showed that 72 patients with JIA had significant depression. Of these 72 patients, 50 (69.4%) had mild, 21 (29.2%) had moderate, and one (1.4%) had severe disability according to CHAQ-DI criteria. Age was the only effect modifier significantly associated with significant depression in patients with JIA (P < 0.05).ConclusionA cross sectional survey was carried out to find prevalence of depression in children with JIA. Physical disability and depression were measured using standardized tools. The percentage of significant depression among children with JIA is very high in our local population and was significantly associated with disease severity. Our findings emphasize the need to initiate early and prompt measures to prevent depression and reduce overall morbidity in patients with JIA.

Intravenous abatacept in Japanese patients with polyarticular-course juvenile idiopathic arthritis: results from a phase III open-label study

BackgroundTo investigate efficacy and safety of intravenous abatacept in Japanese patients with active polyarticular-course juvenile idiopathic arthritis (pJIA).MethodsIn this phase III, open-label, multicenter, single-arm study, patients with pJIA aged 4–17 years who failed ≥1 biologic or methotrexate received weight-tiered (< 75 kg: 10 mg/kg; 75–100 kg: 750 mg; > 100 kg: 1000 mg) intravenous abatacept at Weeks 0, 2, 4, and every 4 weeks thereafter. The study comprised a short-term period (16 weeks) and ongoing long-term period. Primary endpoint: Week 16 JIA-American College of Rheumatology criteria 30 (JIA-ACR30) response rate. Secondary endpoints/outcomes included Week 16 JIA-ACR50/70/90 response and inactive disease rates, Childhood Health Assessment Questionnaire-Disability Index (CHAQ-DI), pharmacokinetics, safety, and immunogenicity. Proportions of patients achieving Juvenile Arthritis Disease Activity Score in 27 joints using C-reactive protein (JADAS27-CRP) remission (score < 1) and minimal disease activity (MDA; score < 3.8), were among exploratory endpoints.ResultsAll 20 patients who received study medication completed the short-term period. During the long-term period, two patients discontinued due to insufficient efficacy or patient decision. Median age and disease duration at baseline were 10.5 and 0.75 years, respectively. Week 16 JIA-ACR30 response rate (primary endpoint) was 90.0% (18/20). JIA-ACR50/70/90 response and inactive disease rates at Week 16 were 75.0% (15/20), 70.0% (14/20), 35.0% (7/20), and 25.0% (5/20), respectively. At Week 52, JIA-ACR30/50/70/90 response and inactive disease rates were observed by 88.9% (16/18), 88.9% (16/18), 83.3% (15/18), 66.7% (12/18) and 44.4% (8/18), respectively. CHAQ-DI improved after Week 12. JADAS27-CRP remission and MDA were achieved by 15.0% (3/20) and 45.0% (9/20) of patients at Week 16, and by 50.0% (9/18) and 78.0% (14/18) of patients at Week 52, respectively. The mean abatacept pre-dose serum concentration was above the target therapeutic exposure (10 μg/ml) from Week 8 through Week 16. All adverse events were of mild/moderate intensity, except for one case of severe gastroenteritis. No deaths, malignancies, or autoimmune disorders were observed. No antidrug antibodies were detected through Week 16; one patient had a positive immunogenic response during the cumulative period.ConclusionIntravenous abatacept was efficacious and well tolerated in Japanese patients with active pJIA.Trial registrationClinicalTrials.gov: NCT01835470. Date of registration: April 19, 2013.

The correlation between the Childhood Health Assessment Questionnaire and disease activity in juvenile idiopathic arthritis.

The Childhood Health Assessment Questionnaire (CHAQ) has been adapted from the Stanford Health Assessment Questionnaire for assessing functional ability in children. The present study aimed to determine the correlation between CHAQ and disease activity in juvenile idiopathic arthritis (JIA) during active and inactive disease. JIA patients in the Pediatric Department, Ramathibodi Hospital, between January 2011 and December 2013, were included in the study. The CHAQ disability index (DI) and disease activity variables, including active and limited joint count, erythrocyte sedimentation rate, patient's global assessment (PtGA), physician's global assessment (PGA) and 27-joint Juvenile Arthritis Disease Activity Score (JADAS27), were collected from medical records for each patient over six visits. At each visit, each patient was classified as having either active or inactive disease. The correlations between CHAQ-DI and disease activity variables were analysed using Spearman's correlation. The classification of 139 JIA patients consisted of enthesitis-related arthritis (30.9%), systemic JIA (28.1%), oligoarthritis (16.5%), rheumatoid factor (RF)-negative polyarthritis (15.1%), RF-positive polyarthritis (6.5%) and undifferentiated arthritis (2.9%). Out of 812 patient visits, 606 were in active disease and 206 were in inactive disease. RF-negative polyarthritis had the highest CHAQ-DI (0.39 ± 0.66), while oligoarthritis had the lowest (0.20 ± 0.32). There was a good correlation between CHAQ-DI and JADAS27, PGA and PtGA in all JIA subtypes (p < 0.05) during active disease, but a poor correlation between CHAQ-DI and disease activity variables during inactive disease. CHAQ-DI had a good correlation with disease activity during active disease but a poor correlation during inactive disease. Therefore, CHAQ is only useful for assessing functional ability during active disease.

OP0181 Treating to Target of Minimal Disease Activity and Normal Function in Polyarticular Juvenile Idiopathic Arthritis with Adalimumab: Analysis from A Phase 3 Clinical Trial

BackgroundThe Juvenile Arthritis Disease Activity Score (JADAS)1 is becoming widely accepted in juvenile idiopathic arthritis (JIA) for defining a treat to target strategy.ObjectivesTo evaluate the percentage of patients (pts) treated...

Impact of a 2-yr anti-TNF treatment on the quality of life in JIA patients: a parent's/patient’s view

Background The use of biologic factors in the management of Juvenile Idiopathic Arthritis (JIA) has changed dramatically the course and outcome of the disease. They control the disease symptoms and hinder the joint damage which may provoke major functional limitations, thus affecting the patients’ Health Related Quality of Life (HRQoL). Aim To evaluate changes in the HRQoL variables in patients with JIA, under anti-TNF treatment. Methods 49 patients with refractory to conventional therapy JIA, aged 2-18 years, were studied. They were receiving either Etanercept or Adalimumab for the first time or they were switchers from one anti-TNF to the other one (10/49). All patients were clinically and laboratory assessed pre-treatment and thereafter every 6 months, until the completion of 2 years. At the same timepoints, the following variables were collected: the Childhood Health Assessment Questionnaire (CHAQ) Disability Index (DI), the patient’s/parent’s global assessment of pain and wellbeing (P/PVAS) and the physician’s global assessment (MDVAS) through the visual analogue scale (VAS). Results During anti-TNF treatment, all the above mentioned core set variables P/PVAS showed a significant improvement (p<0.05). Similarly, the improvement of MDVAS was positively correlated with the improvement of CHAQ DI (rsq=0.43), of VAS pain (rsq=0.66) and of VAS wellbeing (rsq=0.64). No correlation between VAS wellbeing and JIA duration was found. Pain was the only independent factor related with a poor score of wellbeing. Conclusion This study shows that all variables associated with HRQoL in patients with JIA can be substantially improved after 2 years of anti-TNF treatment.

Predictors of poor response to methotrexate in polyarticular-course juvenile idiopathic arthritis: analysis of the PRINTO methotrexate trial

ObjectivesTo determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis.MethodsPatients newly treated for 6 months with MTX enrolled in the...

Does removal of aids/devices and help make a difference in the Childhood Health Assessment Questionnaire disability index?

Objective:To assess whether the removal of aids/devices and/or help from another person in the Childhood Health Assessment Questionnaire (C-HAQ) leads to a significant change in the disability index (DI) score...

Examining the psychometric characteristics of the Dutch childhood health assessment questionnaire: room for improvement?

The aim of this study was to examine the psychometric characteristics of the childhood health assessment questionnaire-disability index (CHAQ-DI). Seventy-six patients with juvenile idiopathic arthritis (JIA), age range 4.8-15.8 years, completed a CHAQ questionnaire one or more times. In total, 321 CHAQ questionnaires were available for analysis. Factor analysis and correlation were used to analyse the data. The analysis indicated that 12 items could be removed from the original 30 items of the CHAQ-DI. Also the addition of "aids and assistance" to the overall scoring method of the CHAQ-DI did not contribute to the overall measuring concept of the CHAQ-DI. The psychometric characteristics of the CHAQ-DI could be improved by removing 12 items from the original 30 items. Moreover, a simple scoring method, without the addition of aids and assistance to the total CHAQ-DI improves sensitivity to change of the CHAQ-DI.

Validity of the childhood health assessment questionnaire is independent of age in juvenile idiopathic arthritis.

To determine whether the Childhood Health Assessment Questionnaire (CHAQ) is valid for the comparison of different age subgroups and for longitudinal studies in juvenile idiopathic arthritis (JIA). A CHAQ was administered to 306 children with JIA. Rasch analyses were used to compare the difficulty of each of the 30 items of the questionnaire for children of 2 age groups (> or =10 years old and <10 years old). Independent of the physical disability level assessed by the Rasch model, 8 of the 30 items (27%) of the CHAQ were rated significantly different in the 2 age groups. Despite this age-related variation in item difficulty, the impact on the CHAQ disability index using its original scoring system remained low (about 0.25 points on a scale of 0-3). The difficulty of 8 of 30 items of the CHAQ depends on the respondent's age. Nevertheless, the design of the CHAQ and its scoring system remove most of the expected physical development bias.