Childhood Health Assessment Questionnaire Score Research Articles

Markers associated with the development of osteoporosis in children with juvenile idiopathic arthritis

Background. The purpose of the study was to investigate the association between bone mineralization le­vel in children with juvenile idiopathic arthritis (JIA) and various exogenous and endogenous factors. Materials and ­me­thods. The study group consisted of 162 children diagnosed with JIA. Medical history, clinical features, and treatment of JIA were studied. Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score (JADAS-27), health status — with the Childhood Health Assessment Questionnaire (CHAQ). Complete blood count with erythrocyte sedimentation rate, biochemical analysis (C-reactive protein, total and ionized calcium) were performed, and instrumental methods (ultrasound densitometry, X-ray and ultrasound examination of joints) were used. Serum levels of osteocalcin, 25-hydroxyvitamin D (25(OH)D), and parathyroid hormone were evaluated using an enzyme-linked immunosorbent assay on an ER500 analyzer (Sinnowa, China). Results. According to our study, osteoporosis was observed in 19 patients (11 girls and 8 boys) out of 162 children with JIA, accounting for 11.7 %. It was significantly more frequent in patients with polyarthritis (73.7 %) compared to those with oligoarthritis (26.3 %) (p < 0.05). Conclusions. Osteoporosis can develop in children with JIA who do not receive systemic glucocorticoid therapy. The main markers associated with osteoporosis in children with JIA include non-infectious disease onset trigger, cervical joint involvement at disease onset, involvement of more than 3 joint groups at onset, gait disturbances, high JADAS-27 and CHAQ scores, signs of systemic inflammatory process, including elevated erythrocyte sedimentation rate.

Safety and efficacy of monthly high-dose vitamin D3 supplementation in children and adolescents with sickle cell disease.

Little is known about the impact of vitamin D supplementation on hand grip strength (HGS) and health-related quality of life (HRQoL) in children and adolescents with sickle cell disease (SCD). We aimed to evaluate the safety and efficacy of monthly high-dose vitamin D3 supplementation and its implications on bone mineral density (BMD), HGS, and HRQoL in patients with SCD and healthy controls. The study included 42 children with SCD and 42 healthy matched controls. The study participants were supplemented with high-dose monthly oral vitamin D3. Changes in the serum level of 25(OH) vitamin D3, maximum HGS, and BMD from baseline to 6months were assessed, and the HRQoL questionnaire and Childhood Health Assessment Questionnaire (CHAQ) were used to evaluate the functional capacity. At baseline, SCD subjects had poorer growth status indicated by negative Z scores. Suboptimal BMD was detected by significantly lower Z score, and lower HGS and worse HRQL parameters were found compared to the controls (P < 0.001). Median 25(OH) vitamin D3 was significantly lower in SCD patients compared to controls (16.5 vs. 28ng/mL, respectively (P < 0.001)). After 6months of vitamin D supplementation, there was significant improvement in the DEXA Z-score (P < 0.001), limitation of physical health (P = 0.02), pain scores (P < 0.001), and CHAQ grades (P = 0.01) in SCD patients. A significant improvement in HGS (P < 0.001 and P = 0.005) as well as the CHAQ score (P < 0.001 and P = 0.003) was detected in the SCD group and controls, respectively. There were no reported clinical adverse events (AEs) or new concomitant medications (CMs) during the study duration, and safe levels of Ca and 25 (OH) D3 were observed at 3 and 6months for both groups. There was a significant positive correlation between HGS and total physical score (r = 0.831, P < 0.001) and a negative correlation with CHAQ score (r = - 0.685, P < 0.001). We also detected a significant positive correlation between vitamin D levels at 6months and HGS (r = 0.584, P < 0.001), pain score (r = 0.446, P < 0.001), and a negative correlation with CHAQ score (r = - 0.399, P < 0.001). Conclusion: Monthly oral high-dose vitamin D supplementation was safe and effective in improving vitamin D levels, HGS, and HRQoL in SCD children and healthy subjects, and BMD scores in SCD patients. Further randomized controlled trials are warranted to assess an optimal dosing strategy and to investigate the impact on clinically significant outcomes in children and adolescents with SCD and their healthy counterparts. Trial registration: ClinicalTrials.gov , identifier NCT06274203, date of registration: 23/02/2024, retrospectively registered. What is known: • Several studies have reported a high prevalence of vitamin D deficiency and suboptimal bone mineral density (BMD) in sickle cell disease (SCD) patients. • Musculoskeletal dysfunction is reported in SCD patients with a negative impact on physical activity and health-related quality of life (HRQL). • Little is known regarding the impact of vitamin D3 supplementation in children and adolescents with SCD. What is new: • We found that monthly oral high-dose vitamin D3 supplementation was safe, tolerated, and effective in improving serum vitamin D levels, HGS, BMD scores, and HRQL in SCD patients.

Real-World Health Care Outcomes and Costs Among Patients With Juvenile Idiopathic Arthritis in Spain

Background: Juvenile idiopathic arthritis (JIA) is the most frequent chronic rheumatic disease in children. If inflammation is not adequately treated, joint damage, long-term disability, and active disease during adulthood can occur. Identifying and implementing early and adequate therapy are critical for improving clinical outcomes. The burden of JIA on affected children, their families, and the healthcare system in Spain has not been adequately assessed. The greatest contribution to direct costs is medication, but other expenses contribute to the consumption of resources, negatively impacting healthcare cost and the economic conditions of affected families. Objective: To assess the direct healthcare, indirect resource utilization, and associated cost of moderate-to-severe JIA in children in routine clinical practice in Spain. Methods: Children were enrolled in this 24-month observational, multicentric, cross-sectional, retrospective study (N = 107) if they had been treated with biologic disease-modifying anti-rheumatic drugs (bDMARDs), had participated in a previous study (ITACA), and continued to be followed up at pediatric rheumatology units at 3 tertiary Spanish hospitals. Direct costs included medication, specialist and primary care visits, hospitalizations, emergency visits or consultations, surgeries, physiotherapy, and tests. Indirect costs included hospital travel expenses and loss of caregiver working hours. Unitary costs were obtained from official sources (€, 2020). Results: Overall, children had inactive disease/low disease activity according to JADAS-71 score and very low functional disability as measured by Childhood Health Assessment Questionnaire score. Up to 94.4% of children received treatment, mainly with bDMARDs as monotherapy (84.5%). Among anti-TNFα treatments, adalimumab (47.4%) and etanercept (40.2%) were used in similar proportions. Annual mean (SD) total JIA cost was €7516.40 (€5627.30). Average cost of pharmacological treatment was €3021.80 (€3956.20), mainly due to biologic therapy €2789.00 (€3399.80). Direct annual cost (excluding treatments) was €3654.60 (€3899.00). Indirect JIA cost per family was €747.20 (€1452.80). Conclusion: JIA causes significant costs to the Spanish healthcare system and affected families. Public costs are partly due to the high cost of biologic treatments, which nevertheless remain an effective long-term treatment, maintaining inactive disease/low disease activity state; a very low functional disability score; and a good quality of life.

Real-World Health Care Outcomes and Costs Among Patients With Juvenile Idiopathic Arthritis in Spain.

Background: Juvenile idiopathic arthritis (JIA) is the most frequent chronic rheumatic disease in children. If inflammation is not adequately treated, joint damage, long-term disability, and active disease during adulthood can occur. Identifying and implementing early and adequate therapy are critical for improving clinical outcomes. The burden of JIA on affected children, their families, and the healthcare system in Spain has not been adequately assessed. The greatest contribution to direct costs is medication, but other expenses contribute to the consumption of resources, negatively impacting healthcare cost and the economic conditions of affected families. Objective: To assess the direct healthcare, indirect resource utilization, and associated cost of moderate-to-severe JIA in children in routine clinical practice in Spain. Methods: Children were enrolled in this 24-month observational, multicentric, cross-sectional, retrospective study (N = 107) if they had been treated with biologic disease-modifying anti-rheumatic drugs (bDMARDs), had participated in a previous study (ITACA), and continued to be followed up at pediatric rheumatology units at 3 tertiary Spanish hospitals. Direct costs included medication, specialist and primary care visits, hospitalizations, emergency visits or consultations, surgeries, physiotherapy, and tests. Indirect costs included hospital travel expenses and loss of caregiver working hours. Unitary costs were obtained from official sources (€, 2020). Results: Overall, children had inactive disease/low disease activity according to JADAS-71 score and very low functional disability as measured by Childhood Health Assessment Questionnaire score. Up to 94.4% of children received treatment, mainly with bDMARDs as monotherapy (84.5%). Among anti-TNFα treatments, adalimumab (47.4%) and etanercept (40.2%) were used in similar proportions. Annual mean (SD) total JIA cost was €7516.40 (€5627.30). Average cost of pharmacological treatment was €3021.80 (€3956.20), mainly due to biologic therapy €2789.00 (€3399.80). Direct annual cost (excluding treatments) was €3654.60 (€3899.00). Indirect JIA cost per family was €747.20 (€1452.80). Conclusion: JIA causes significant costs to the Spanish healthcare system and affected families. Public costs are partly due to the high cost of biologic treatments, which nevertheless remain an effective long-term treatment, maintaining inactive disease/low disease activity state; a very low functional disability score; and a good quality of life.

The validity and reliability of the Juvenile Arthritis Functional Assessment Report (JAFAR) in children/adolescents with Juvenile Idiopathic Arthritis: The Turkish version study

The aim of the study was to describe the validity and reliability of the Turkish version of Juvenile Arthritis Functional Assessment Report (JAFAR) in children/adolescents with Juvenile Idiopathic Arthritis (JIA). Sixty-nine children/adolescents with JIA were included in the study. JAFAR(TR)-Child and Parent forms were applied to the patients with JIA and to their parents for test retest at one-week intervals, the patients did not receive additional treatment and his/her pharmacological treatment did not change for that week. Test-retest reliability was evaluated by intraclass correlation coefficient (ICC), and internal consistency reliability of multi-item subscales was evaluated by calculating Cronbach's alpha coefficient. Correlations between JAFAR(TR)-Child and Parent with the Pediatric Quality of Life Inventory 3.0. Module Arthritis (PedsQL), the Childhood Health Assessment Questionnaire (CHAQ), and the Juvenile Arthritis Disease Activity Score (JADAS) were evaluated to determine construct validity. The ICC value for the test/retest reliability of JAFAR(TR)-Child was 0.963 and of JAFAR(TR)-Parent was 0.576. JAFAR(TR)-Child total score had low to moderate correlations with PedsQL Child (r=-0.34; p=0.004), CHAQ (r=0.40; p=0.001), and JADAS total score (r=0.42; p=0.000). JAFAR(TR)-Parent total score had moderate to high correlations with PedsQL Parent (r=-0.55; p=0.000), CHAQ (r=0.72; p=0.000) and JADAS total score (r=0.53; p= 0.000). The Turkish version of JAFAR was found to be clinically valid and reliable in JIA.

P007 Novel integration of a digital patient-reported outcome measure into an NHS electronic health record

Abstract Background/Aims The Childhood Health Assessment Questionnaire (CHAQ) and 0-10cm visual analogue scale (VAS) for self-reporting global-wellbeing form part of the Core Outcome Variables measuring disease activity in juvenile idiopathic arthritis. However, reported barriers to using paper-based CHAQs include: impacts on consultation time and issues with scoring and transcribing CHAQ data into health records. To address these issues we aimed to develop a digital CHAQ (eCHAQ) for patients attending paediatric rheumatology clinics at Alder Hey Children’s Hospital. Additional objectives included: developing automated invitations to complete eCHAQs; achieving automated scoring and integration of CHAQ data into the hospital’s Electronic Health Record (EHR); investigating the acceptability of the eCHAQ to patients and clinical staff; and studying the effects of the system on CHAQ completion rates. Methods The eCHAQ was developed collaboratively by the Alder Hey Information Technology team, clinicians, and a technology provider, Aire Logic. Acceptability was measured using a patient survey (patient and parent ePROM questionnaire), and a survey (Technology Acceptance Model 2 questionnaire) and focus group involving clinical staff. CHAQ completion rates were measured using a before- after study of data from clinical records. Results The eCHAQ was implemented in April 2021 and approximately 500 eCHAQ invitations are sent monthly. Technical features include automated generation of SMS invitations to complete eCHAQs; automated scoring of CHAQ assessments; and unique URLs (webpage addresses) enabling automated re-integration of CHAQ data into the EHR. Patients/care-givers (n = 24) scored the eCHAQ highly across Likert scale items designed to assess accessibility (median response = 5 [strong agreement]; interquartile range [IQR] = 0); and acceptability (median response = 5 [strong agreement]; IQR = 0). Clinical staff (n = 7; consultants, nurse specialists and occupational therapists) also scored the eCHAQ highly across domains tested using Likert scale items including “Perceived Usefulness” (median response = 7 [strong agreement]; IQR = 0.25) and “Output Quality” (median response = 7 [strong agreement]; IQR = 1). Thematic analysis of a focus group discussion involving 10 members of the Rheumatology MDT identified that strengths of the eCHAQ system included improved access to clinical data, improved data quality and time saved. Concerns relating to access to digital technologies were highlighted. Use of the eCHAQ resulted in a significant increase in the CHAQ completion rate. Before the implementation of the eCHAQ, documentation of a CHAQ score in the EHR occurred in 33/100 (33%) assessed records compared to 47/65 (72%) after the introduction of the eCHAQ system (chi-square = 11.51; p &amp;lt; 0.05). Conclusion Development of a highly-automated digital CHAQ, with integration directly into an NHS EHR, was technically feasible. Introduction of the eCHAQ was found to be highly acceptable to patients and clinical staff and was associated with a significant improvement in CHAQ completion rates. Disclosure M. Neame: None. D. Reilly: None. D. Bond: Other; Employee of private technology provider involved in the development of the reported intervention. A. Puthiyaveetil: None. L. McCann: None. K. Mahmood: None. B. Almeida: None. C.E. Pain: None. V. Furfie: None. G. Cleary: None.

Anxiety and Depressive Symptoms in Juvenile Idiopathic Arthritis Correlate With Pain and Stress Using PROMIS Measures

Describe anxiety and depressive symptoms in children with juvenile idiopathic arthritis (JIA) using Patient Reported Outcomes Measurement Information System (PROMIS) measures and evaluate potential correlations with disease manifestations. We performed a cross-sectional study of children with JIA and a parent proxy who completed PROMIS measures on depression, anxiety, stress, and pain. The Childhood Health Assessment Questionnaire (CHAQ) measured mobility, and the clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10) measured disease activity. Eighty-four patients completed the study. Demographic median values included age (14 yrs), disease duration (4.73 yrs), CHAQ score (0), total active joint count (0), and cJADAS10 (2). Using cJADAS10, 57 patients (70%) had inactive or low disease activity. Mean PROMIS t-scores for depressive and anxiety symptoms were lower in children with JIA compared to the reference population (P < 0.0001). Nineteen patients (23%) had moderate to severe symptoms of anxiety and/or depression. Age and CHAQ score (mobility) correlated with depressive symptoms (r = 0.36, P =0.0008 and r = 0.32, P = 0.0029, respectively) but not anxiety. Depressive and anxiety symptoms correlated with pain (r = 0.64 and r = 0.47, respectively, P < 0.0001) and stress (r = 0.79 and r = 0.75, respectively, P < 0.0001) but not with sex, JIA subtype, disease duration, or disease activity. Approximately one-quarter of children with JIA reported moderate to severe symptoms of anxiety and depression. These symptoms are associated with pain and stress, but they are not associated with other disease manifestations. Understanding how mental health symptoms and JIA affect each other is necessary in order to improve patient outcomes and provide well-rounded care.

Common Functional Ability Score for Young People With Juvenile Idiopathic Arthritis.

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.

Evaluation of serum ferritin and its correlation with disease activity in non-systemic juvenile idiopathic arthritis

Background: Serum ferritin is considered as an acute phase reactant that often increases in presence of an active inflammation. Its status as a disease activity marker is well-established in systemic juvenile idiopathic arthritis (JIA), but underexplored in other categories. The objective of this study was to evaluate the relation of serum ferritin with disease activity in non-systemic categories of JIAMethods: A prospective analytical study was carried out involving 46 JIA patients (diagnosed and categorized on the basis of ILAR criteria) with high disease activity based on juvenile arthritis disease activity score27 (JADAS27). childhood health assessment questionnaire (CHAQ), erythrocyte sedimentation rate (ESR), serum ferritin, pain VAS (visual analog scale), parent global VAS, JADAS27 were measured at initial visit, six months and one year.Results: 40 (21 female, 19 male) out of 46 patients completed follow-up of 1 year. Amongst them, 11 patients had systemic arthritis, 10 had oligoarthritis, 11 had RF positive polyarthritis and 8 had RF negative polyarthritis. Their median ages at the initial visit were 7, 6.5, 8 and 7.5 years respectively. Serum ferritin, CRP, ESR, CHAQ score and JADAS decreased over time in all four categories of JIA. Median ESR, ferritin, CHAQ and JADAS27 were higher in systemic JIA compared to other groups in all three visits. Serum ferritin significantly correlated with JADAS27 at all three visits in systemic arthritis; at initial visit in oligoarthritis; at initial visit and 6 months in both RF positive and RF negative polyarthritis.Conclusions: In patients with systemic JIA, serum ferritin showed significant correlation throughout disease course even when the disease activity was low. But in non-systemic categories of JIA serum ferritin had a significant positive correlation with disease activity only when the disease activity was high.

Effects of sense and functionality changes in the hands on activity and participation in patients with juvenile scleroderma

Objectives The purpose of the study was to examine the effects of sense and functionality changes in the hands on activity and participation in patients with juvenile scleroderma (JS). Methods Sixteen patients with juvenile localized scleroderma (JLS), 14 patients with Juvenile Systemic Sclerosis (JSS), and 30 healthy controls were included. Light touch-deep pressure sensation was assessed by Semmes-Weinstein monofilament test (SWMT). Localization sensation testing was performed by lightly stroking the patient’s skin. The hand joint range of motion was measured with a goniometer, hand grip strength with Dynomometer, the pinch gripping force with pinch meter, and the hand mobility with modified Hand Mobility in Scleroderma (mHAMIS). Children completed their activity and participant performance status with ‘Childhood Health Assessment Questionnaire (CHAQ)’ and ‘Jebson Taylor Hand Function Test (JTHFT)’ questionnaire tests. The quality of life was evaluated using the ‘Scleroderma Health Assessment Questionnaire (SHAQ)’. Results There were significantly differences among evaluated three groups in light of touch-deep pressure sensation, sense of touch localization, range of motion, mHAMIS scores, JTHFT scores, all CHAQ scores, and almost all SHAQ score (p < .05). Over than half of patients with JSS (57.1%) and almost half of patients with JLS stated that their diseases obstructed them from doing any activity (p < .001). A significant percent of JSS patients (64.3%) had hand and wrist joint involvement. Conclusion Sensory and functional disorders caused by hand involvement in JS patients result in limitation of daily living activities and affect negatively the effective usage of the hand. Approximately half of the JS patients had disabilities in performing pinch motor skills of hands. The assessment of sensory symptoms that affect the functionality, activity level and participation of JSS and JLS patients should be considered during the routine clinical examination. We suggest the sensory therapies as an important factor in increasing the effectiveness of rehabilitation.

Upper extremity rehabilitation using video games in cerebral palsy: a randomized clinical trial.

The aim of the present study was to compare the effects of neurodevelopmental therapy (NDT)-based upper extremity rehabilitation and video game-based therapy (VGBT) using Nintendo® wii and leap motion controller (LMC) games on upper extremity function in patients with cerebral palsy (CP). The 30 patients included in the present study were randomized to two groups: VGBT group (VGBT using Nintendo® Wii and LMC games) and control group (NDT-based upper extremity rehabilitation). Both groups trained 3days a week for 8weeks. Manual dexterity was evaluated using the "Minnesota Manual Dexterity Test (MMDT)", functional ability using the "Childhood Health Assessment Questionnaire (CHAQ)" and the "Duruoz Hand Index (DEI)" and grip and pinch strengths using a dynamometer. Following treatment, significant changes were found, MMDT, grip and pinch strength, CHAQ, and DHI scores in both groups (p < 0.05); however, VGBT group was statistically superior to group II with respect to changes in MMDT (p < 0.05). VGBT using Nintendo®Wii and LMC games had slightly superior effects on manual dexterity in patients with CP while compared with NDT-based upper extremity rehabilitation. Futhermore, the effects of both treatment programs on grip strengths and functional ability were similar beneficial.

The effects on Oral Related Quality of Life induced by periodontitis in patients with juvenile idiopathic arthritis

The objective of study was to evaluate the impact of periodontitis on the health-related quality of life (HRQoL) in patients affected by juvenile idiopathic arthritis (JIA). Sixty consecutive patients with JIA with or without periodontitis and 33 healthy control subjects were enrolled in the study. The demographic data, disease activity and clinical characteristics were obtained from all patients. HRQoL and disability was assessed by two questionnaires, the Italian version of the Childhood Health Assessment Questionnaire (C-HAQ) and the Child Perception Questionnaire (CPQ11-14). Possible determining factors of periodontitis comprised demographic and disease characteristics and HRQOL scores that were assessed by uni- and multivariable logistic regression analysis. Compared to patients without periodontitis, JIA patients with periodontitis presented higher disability and lower HRQOL scores. The multivariable logistic regression analysis highlighted that female subjects (OR 1.4) or a JIA duration over 4.1 years (OR 2.6), higher C-HAQ and CPQ11-14 scores (OR 2.8 – 2.9) were the greatest determining factors for periodontitis. JIA patients with periodontitis presented lower HRQoL compared to JIA patients without periodontitis. Periodontitis was strongly associated with JIA duration and activity and negatively influenced the HRQoL of JIA patients.

Enthesitis-related Arthritis: Prevalence and Complications of Associated Uveitis in Children and Adolescents From a Population-based Nationwide Study in Germany

Enthesitis-related arthritis (ERA) represents a subgroup of juvenile idiopathic arthritis (JIA) that is regularly accompanied by anterior uveitis. This study describes the prevalence and characteristics of ERA-related uveitis. Cross-sectional data from the National Pediatric Rheumatological Database (NPRD) were used to characterize ERA-related uveitis (ERA-U). In addition to sociodemographic variables, we documented the occurrence of uveitis and course of disease, including symptoms, visual acuity, and complications, as well as JIA characteristics such as disease activity (Juvenile Arthritis Disease Activity Score 10), functional ability (Childhood Health Assessment Questionnaire score), laboratory variables, and treatment. In the years from 2002 to 2014, there were 3778 (15.2%) of a total of 24,841 JIA patients recorded in the NPRD who had ERA, and 280 (7.4%) of them had developed uveitis. Detailed ophthalmological documentation by a uveitis add-on module was available for 22.9% of these patients. Uveitis onset was acutely symptomatic in 63% of patients. Patients with uveitis were more frequently male, HLA-B27-positive, younger at ERA onset, and they had higher erythrocyte sedimentation rate values at first uveitis documentation than those without uveitis. Uveitis was diagnosed at a mean age of 11.5 (± 3.9) years (50% within 2 years after ERA onset). Systemic treatment with corticosteroids and synthetic and biologic disease-modifying antirheumatic drugs was associated with a (not significantly) lower risk of developing uveitis. The course of disease in ERA-U patients is frequently similar to HLA-B27-associated uveitis in adults; however, a subgroup of patients presents with asymptomatic uveitis.

Worse Quality of Life, Function, and Pain in Children With Enthesitis, Irrespective of Their Juvenile Arthritis Category.

To estimate the impact of enthesitis on patient-reported outcomes in children with juvenile idiopathic arthritis (JIA), irrespective of JIA category. Children enrolled in the Research in Arthritis in Canadian Children Emphasizing Outcomes cohort were studied. Entheseal tenderness by physician examination in 33 defined locations, Juvenile Arthritis Quality of Life Questionnaire (JAQQ), Quality of My Life (QoML) Questionnaire, Childhood Health Assessment Questionnaire (C-HAQ), and a pain visual analog scale were completed at enrollment, every 6 months for 2 years, and then yearly up to 5 years. Analyses consisted of descriptive statistics, linear mixed models for longitudinal data, and analysis of covariance. Among 1,371 patients followed for a median of 35.3 months (interquartile range 22.1, 49.2), 214 (16%) had enthesitis, of whom 137 (64%) were classified as having enthesitis-related arthritis. After adjusting for JIA category and covariates, children with enthesitis reported higher JAQQ (mean raw score 2.71 versus 2.16, adjusted difference 0.41 points; 95% confidence interval [95% CI] 0.22, 0.59), higher C-HAQ (0.47 versus 0.31, adjusted difference 0.14 points; 95% CI 0.07, 0.22), higher pain (3.01 versus 1.68, adjusted difference 0.94 points; 95% CI 0.64, 1.25), and lower QoML (7.02 versus 8.23, adjusted difference -0.80 points; 95% CI -1.09, -0.51) scores than children without enthesitis. These differences persisted up to 5 years. Children with enthesitis, regardless of JIA category, report worse patient-reported outcomes than those without enthesitis. Thus, enthesitis should be assessed in all children with JIA.

Correlation of ultrasonography synovitis with disease activity and clinical response to etanercept treatment in juvenile idiopathic arthritis patients.

This study aimed to investigate the correlation of ultrasonography (US) of synovitis with disease activity and clinical response to etanercept (ETN) in juvenile idiopathic arthritis (JIA) patients. Eighty-two JIA patients who underwent ETN treatment for 24 weeks were consecutively enrolled. US evaluations of 28 joints (shoulder, elbow, wrist, metacarpophalangeal, and proximal interphalangeal of hands and knee) at baseline were performed using grey-scale US and power doppler (PD) US, and US synovitis was defined as grey-scale abnormalities or PD abnormalities. Clinical response was assessed according to the ACRpedi 50 response criteria. In total, 2296 joints were scanned and 608 (26.5%) joints presented US synovitis, which was numerically higher than clinical synovitis (513 (22.3%)). The mean number of joints showing synovitis on US was 7.42±3.35, which was also numerically higher than that of clinical synovitis (6.26±2.70). The number of joints showing synovitis on US was positively correlated with C-reactive protein, erythrocyte sedimentation rate, number of joints with active disease, number of joints with limited range of motion, physician's global assessment of disease activity, parent/patient global assessment of overall well-being, and childhood health assessment questionnaire score. Most interestingly, the baseline number of joints showing synovitis on US was increased in ACRpedi 50 response JIA patients compared to non-response JIA patients, and it serves as an independent predictive factor for higher clinical response to ETN treatment. In conclusion, US is a more sensitive test to evaluate subclinical synovitis and disease activity in JIA patients, and US synovitis might serve as a marker for predicting increased clinical response rate to ETN treatment.

Assessment of Health Related Quality of Life (HRQL) in Egyptian Children with Rheumatic Diseases; Its Relation to Disease Activity and Functional Disability

Background Many pediatric rheumatic diseases persist into adulthood, with negative squeal from the disease or its treatment. We aimed to assess health related quality of life in a group of children with rheumatic diseases and its relation to disease activity and functional disability. Materials and Methods Fifty one patients were divided into three groups: Group 1 includes 27 patients diagnosed as juvenile idiopathic arthritis (JIA), Group 2 includes 15 patients diagnosed as juvenile onset systemic lupus erythematosus (SLE) and Group 3 includes nine patients; three diagnosed as Juvenile Dermatomyositis, three diagnosed as Familial Mediterranean Fever and three female’s patients diagnosed as Juvenile Scleroderma. Childhood Health Assessment Questionnaire (CHAQ), Pediatric Quality of Life generic core scale version 4.0, Visual analogue scale for pain and Visual analogue scale (VAS) for global assessment were recorded. The activity index was assessed in each patient according to the nature of the disease. Results In JIA patients, the mean PedsQL score was 73.6+ 15.4, the mean CHAQ score was 0.7+ 0.7, the mean DAS28 was 3.5+ 0.9, with a significant negative correlation between PedsQL and CHAQ (p=0.48), VAS pain (p=0.001), and DAS 28 activity index (p=0.017). In SLE patients, the mean PedsQL was 66.4 + 20.3, mean CHAQ score was 0.7 + 0.67 and mean SLEDAI-2K was 24.2 + 14.6 with no significant correlation between functional disability and SLEDAI-2K (p=0.539). PedsQL showed a significant negative correlation with SLEDAI-2k (p=0.001), and positive correlation between CHAQ (p=0.022). Conclusion Health related quality of life in patients with juvenile rheumatic diseases is correlated with disease activity and functional disability and should be assessed in regular basis.

Treat to target (drug-free) inactive disease in DMARD-naive juvenile idiopathic arthritis: 24-month clinical outcomes of a three-armed randomised trial

QuestionWhich is the best strategy to achieve (drug-free) inactive disease in juvenile idiopathic arthritis (JIA)?MethodsIn a randomised, single-blinded, study in disease-modifying anti-rheumatic drug (DMARD)-naive patients with JIA, three treatment-strategies were...

The Assessment of Serum Endocan Levels in Children With Juvenile Idiopathic Arthritis.

This study aims to evaluate the levels of serum endocan in children with juvenile idiopathic arthritis (JIA). Sixty-seven children with JIA (30 males, 37 females; mean age 10.4±4.9 years; range 2 to 18 years) and a sex- and age- matched healthy control group of 39 children (16 males, 23 females; mean age 9.3±4.1 years; range 1 to 17 years) were recruited. Patients with JIA were divided into two groups as the clinically active JIA group (n=27) and inactive JIA group (n=40). The median serum endocan level in patients with JIA was significantly higher than in the control group (633.75 ng/L vs. 379.76 ng/L, p<0.01). Comparison between patients with active JIA and inactive JIA was not significant in terms of endocan levels (618.70 ng/L vs. 687.36 ng/L, p=0.34). There was a weak negative correlation between Childhood Health Assessment Questionnaire scores of patients with JIA and serum endocan levels. The high level of serum endocan highlighted the endothelial damage in patients with JIA.

The impact of temporomandibular joint arthritis on functional disability and global health in patients with juvenile idiopathic arthritis.

The objective of the study was to evaluate the impact of temporomandibular joint (TMJ) arthritis on the functional disability and quality of life in patients affected by juvenile idiopathic arthritis (JIA). Sixty-two consecutive patients with JIA with or without TMJ arthritis and 35 healthy control subjects were enrolled in the study. The demographic data, disease activity and clinical characteristics were obtained from all patients. The functional disability was assessed using the Italian version of the Childhood Health Assessment Questionnaire (C-HAQ). The oral health-related quality of life (OHRQoL) was assessed using the Child Perception Questionnaire (CPQ11-14). Possible determining factors of TMJ arthritis comprised demographic, disease characteristics and scores derived from questionnaires that were assessed by a uni and multivariable logistic regression analysis. Compared with patients without TMJs arthritis, JIA patients with TMJ arthritis presented higher functional disability. The multivariable logistic regression analysis performed showed that female subjects (OR = 1.5, P = 0.041), with a JIA duration over 3.9 years (OR = 2.7, P = 0.033) and presenting higher C-HAQ and CPQ11-14 scores (OR = 2.7, P = 0.012 and OR = 2.9, P = 0.015, respectively) were the greatest determining factors for TMJ arthritis. JIA patients with TMJ arthritis presented higher functional disability and lower OHRQoL scores compared with JIA patients without TMJ arthritis. TMJ arthritis was strongly associated with JIA duration and activity, especially in female patients.

Healthcare Use Patterns and Economic Burden of Chronic Musculoskeletal Pain in Children before Diagnosis

To evaluate the healthcare use and costs of amplified musculoskeletal pain syndrome (AMPS) in children before diagnosis. We performed a retrospective study in children with AMPS at a pediatric rheumatology clinic between 2010 and 2014. Data were abstracted on 80 patients after primary rheumatic diseases were excluded. Healthcare visits, medications and diagnostic testing that occurred in the years before diagnosis were collected. The Medical Expenditure Panel Survey was used to estimate visit costs. Patients were adolescent females (89%) and white (86%). The median time to diagnosis was 10.2 months. The median pain score was 6.5 and the median Childhood Health Assessment Questionnaire score was 1.1. In this cohort, 29% had at least 1 ED visit and 5% were hospitalized. All patients saw a rheumatologist and 41% had visited another specialist, typically orthopedics and sports medicine. More than one-half had at least 1 radiographic study and 21% had at least 1 magnetic resonance imaging. The total cost for office, emergency department, and hospital visits for AMPS in all 80 patients was $152 853. The mean cost per patient over the entire study period (2008-2014) was $1911 ± $3808, and 43% of costs were outpatient visits. Children with AMPS have high levels of disability and take a long time to be diagnosed. As a result, even before diagnosis, they have high levels of healthcare use, diagnostic testing, and medical costs. Early recognition of disability and quicker referral to trained subspecialists may improve the prognosis, reduce unnecessary testing, and reduce the overall costs of healthcare.