Adherence, helpfulness and barriers to treatment in juvenile idiopathic arthritis - data from a German Inception cohort.

OBJECTIVES: To develop and evaluate German versions of the Parent Adherence Report Questionnaire (PARQ) and Child Adherence Report Questionnaire (CARQ) and to evaluate adherence in patients with juvenile idiopathic arthritis (JIA). METHODS: The PARQ and CARQ were translated into German, cross-culturally adapted and administered to patients (age ≥ 8 years) and their parents enrolled in the Inception Cohort Study of newly diagnosed JIA patients (ICON). The psychometric issues were explored by analyzing their test-retest reliability and construct validity. RESULTS: Four hundred eighty-one parents and their children with JIA (n = 465) completed the PARQ and CARQ at the 4-year follow-up. Mean age and disease duration of patients were 10.1 ± 3.7 and 4.7 ± 0.8 years, respectively. The rate of missing values for PARQ/CARQ was generally satisfactory, test-retesting showed sufficient reliability. PARQ/CARQ mean child ability total scores (0-100, 100 = best) for medication were 73.1 ± 23.3/76.5 ± 24.2, for exercise: 85.6 ± 16.5/90.3 ± 15.0, for splints: 72.9 ± 24.2/82.9 ± 16.5. Construct validity was supported by PARQ and CARQ scores for medications, exercise and splints showing a fair to good correlation with the Global Adherence Assessment (GAA) and selected PedsQL scales. Adolescents showed poorer adherence than children. About one third of the parents and children reported medication errors. Perceived helpfulness was highest for medication, and adverse effects were reported the greatest barrier to treatment adherence. CONCLUSIONS: The German versions of the PARQ and CARQ appear to have a good reliability and sufficient construct validity. These questionnaires are valuable tools for measuring treatment adherence, identifying potential barriers and evaluating helpfulness of treatments in patients with JIA.

Consumer perspective on healthcare services for juvenile idiopathic arthritis: results of a multicentre JIA inception cohort study.

OBJECTIVES: To evaluate healthcare services for patients with juvenile idiopathic arthritis (JIA) from the parent-proxy perspective and to identify factors associated with perceived deficits in care. METHODS: Patients with JIA from 11 paediatric rheumatology units were enrolled in an inception cohort within the first 12 months after diagnosis. Healthcare services were assessed using The Child Healthcare Questionnaire on satisfaction, utilisation and needs. Factors associated with deficits in care were identified by logistic regression analysis. RESULTS: Data from parents of 835 JIA-patients were included in the analysis. At the assessment (4.7 months after diagnosis), 85% of the patients received drug treatment, and 50% had received multi-professional care. The most frequently used services were physiotherapy (84%), occupational therapy (23%), and telephone counselling (17%). Almost one-third of families reported that they had not received the services that they needed, with health education being the most frequently reported need. Most parents (93%) were satisfied with the overall healthcare provided for their children, especially regarding doctors' behaviour. However, approximately 1 in 3 consumers were dissatisfied with the time to JIA diagnosis and the school services. The lower the child's quality of life, the higher the chance was that the child and the family received multi-professional care, perceived unmet needs, and were dissatisfied with care. CONCLUSIONS: According to parents' experience and satisfaction with their child's care, performance at the system level can be further improved by diagnosing JIA earlier, providing additional information at disease onset, and ensuring that the child's social environment is taken into account.

Predictive factors and biomarkers for the 2-year outcome of uveitis in juvenile idiopathic arthritis: data from the Inception Cohort of Newly diagnosed patients with Juvenile Idiopathic Arthritis (ICON-JIA) study.

OBJECTIVE: To define predictors for the 2-year outcome in terms of achieving inactivity, subsequent uveitis reactivation and occurrence of uveitis-related complications of JIA-associated uveitis. METHODS: Demographic and clinical parameters and serum samples of JIA-associated uveitis patients enrolled in ICON at ⩽1 year of JIA diagnosis were collected at study enrolment, every 3 months during the first year and subsequently every 6 months. Predictors for the 2-year outcome were evaluated by linear mixed models. RESULTS: Of 954 JIA patients included, uveitis occurred in 106 up to the first 2-year follow-up, with 98 of them having complete ophthalmological documentation. In 81.8% and 80.0% of patients, uveitis inactivity was achieved at the 1- and 2-year follow-up after uveitis onset, respectively. JIA onset after the age of 5 years, no use of topical corticosteroids, and adalimumab treatment were significantly associated with an inactive uveitis for at least 6 months (n = 57). Correlates for subsequent uveitis reactivation (n = 16, 30.2%) were age at uveitis onset ⩽5 years and active disease (clinical Juvenile Arthritis Disease Activity Score >4.5). Uveitis-related complications were present in 29.8% of patients at first uveitis documentation and in 30.7% and 32.8% at 1- and 2-year follow-up, respectively. Older age at JIA onset, short duration between JIA and uveitis onset, high anterior chamber (AC) cell grades, poor visual acuity, and topical steroid use at first uveitis documentation correlated with uveitis-related complications. CONCLUSION: In addition to demographic risk factors, JIA disease and uveitis activity scores and adalimumab are significant predictors for the 2-year outcome of JIA-associated uveitis patients.

Vitamin D deficiency is associated with higher disease activity and the risk for uveitis in juvenile idiopathic arthritis - data from a German inception cohort.

OBJECTIVE: The objective was to evaluate the 25(OH) vitamin D (25(OH)D) status of patients with juvenile idiopathic arthritis (JIA) and determine whether the 25(OH)D level is associated with disease activity and the course of JIA. METHODS: Patients ≤ 16 years of age with recently diagnosed JIA (< 12 months) were enrolled in the inception cohort of patients with newly diagnosed JIA (ICON), an ongoing prospective observational, controlled multicenter study started in 2010. Clinical and laboratory parameters were ascertained quarterly during the first year and half-yearly thereafter. Of the 954 enrolled patients, 360 patients with two blood samples taken during the first 2 years after inclusion and with follow up of 3 years were selected. The serum 25(OH)D levels were determined and compared with those of subjects from the general population after matching for age, sex, migration status and the month of blood-drawing. RESULTS: Nearly half of the patients had a deficient 25(OH)D level (< 20 ng/ml) in the first serum sample and a quarter had a deficient level in both samples. Disease activity and the risk of developing JIA-associated uveitis were inversely correlated with the 25(OH)D level (ß = - 0.20, 95% CI - 0.37; 0.03, hazard ratio 0.95, 95% CI 0.91; 0.99, respectively). CONCLUSION: In this study, 25(OH)D deficiency was common and associated with higher disease activity and risk of developing JIA-associated uveitis. Further studies are needed to substantiate these results and determine whether correcting 25(OH)D deficiency is beneficial in JIA.

The majority of patients with newly diagnosed juvenile idiopathic arthritis achieve a health-related quality of life that is similar to that of healthy peers: results of the German multicenter inception cohort (ICON).

BACKGROUND: Achieving the best possible health-related quality of life (HRQoL) for a patient is an important treatment goal in juvenile idiopathic arthritis (JIA). We investigated the 36-month trajectories of HRQoL in children with JIA compared with healthy peers and identified the predictors of an unfavorable HRQoL. METHODS: Patients with a recent JIA diagnosis were enrolled in the German inception cohort study ICON. As a peer group, friends of patients of the same age and sex were asked to cooperate. Children were prospectively followed and regularly questioned about their HRQoL using the Pediatric Quality of Life Inventory 4.0 (PedsQL). Disease activity was assessed by the clinical Juvenile Arthritis Disease Activity Score (cJADAS-10), and the burden of the child's chronic illness on their family was assessed by the Family Burden Questionnaire (FaBel). Linear mixed models were used to compare the HRQoL of the patients and their peers. Associations between the health status of a patient at enrollment and an unfavorable HRQoL (PedsQL total < 79.3) at their 3-year follow-up (FU) were analyzed by logistic regression. RESULTS: Data from 953 patients (median symptom duration 6 months, mean age 7.9 years) and 491 healthy peers (aged 8.4 years) were analyzed. During 3 years of FU, the disease activity and HRQoL of the patients improved significantly (cJADAS-10 from 9.8 (6.2) to 2.7 (3.6) and PedsQL total score from 71.7 (18.2) to 87.3 (13.9)). While the HRQoL of the patients varied among the several JIA categories at the time of enrollment, no significant differences were found at the 3-year FU. After 36 months, the HRQoL of the patients had largely converged with that of their healthy peers. JIA patients had a psychosocial health status comparable with their healthy peers, whereas a small significant mean difference remained in physical health (5.8, 95% confidence interval (CI) 4.1-7.6). Up to the 36-month FU, three-quarters of JIA patients attained a favorable HRQoL (PedsQL ≥ 79.3) which was achieved by 90% of the peers. A higher family burden, higher pain level, and lower well-being at enrollment were associated with an unfavorable HRQoL. CONCLUSIONS: Under current therapeutic conditions, an HRQoL corresponding with that of healthy children is a realistic treatment goal in JIA.

Risk Factors and Biomarkers for the Occurrence of Uveitis in Juvenile Idiopathic Arthritis: Data From the Inception Cohort of Newly Diagnosed Patients With Juvenile Idiopathic Arthritis Study.

OBJECTIVE: To analyze the prognostic value of demographic, clinical, and therapeutic factors and laboratory biomarkers and to assess their role in predicting uveitis occurrence in patients with juvenile idiopathic arthritis (JIA). METHODS: Patients with JIA were enrolled within the first year after JIA diagnosis. Demographic and clinical parameters were documented. Serum samples were collected at study enrollment, at 3-month follow-up visits within the first year, and then every 6 months. A multivariable Cox regression analysis was performed to evaluate the impact of demographic, clinical, laboratory, and therapeutic parameters on uveitis onset. RESULTS: We included 954 JIA patients (67.2% female, 54.2% antinuclear antibody [ANA] positive, mean ± SD age at onset 7.1 ± 4.6 years). Uveitis occurred in 133 patients (observation period 44.5 months). Young age at JIA onset and ANA positivity were significantly associated with the onset of uveitis (both P < 0.001). Treatment of arthritis with methotrexate alone (hazard ratio [HR] 0.18 [95% confidence interval (95% CI) 0.12-0.29], P < 0.001) or combined with etanercept (HR 0.10 [95% CI 0.04-0.23], P < 0.001) or adalimumab (HR 0.09 [95% CI 0.01-0.61], P = 0.014) reduced the risk of uveitis onset and the occurrence of uveitis-related complications. Predictors of uveitis onset included elevated erythrocyte sedimentation rate at baseline (HR 2.36 [95% CI 1.38-4.02], P = 0.002) and continuing moderate or high disease activity during follow-up as measured by the 10-joint clinical Juvenile Arthritis Disease Activity Score (HR 4.30 [95% CI 2.51-7.37], P < 0.001). Additionally, S100A12 levels ≥250 ng/ml at baseline were significantly associated with the risk of uveitis (HR 2.10 [95% CI 1.15-3.85], P = 0.016). CONCLUSION: Apart from demographic risk factors and treatment modalities, JIA disease activity scores and laboratory biomarkers could be used to better define the group of JIA patients at high risk of uveitis onset.

The majority of newly diagnosed patients with juvenile idiopathic arthritis reach an inactive disease state within the first year of specialised care: data from a German inception cohort.

OBJECTIVE: To describe the disease characteristics of patients with juvenile idiopathic arthritis (JIA) included in an inception cohort, to analyse how many patients from each JIA category reach an inactive disease state within the first year of specialised care and to determine predictors for attaining inactive disease. METHODS: Patients with JIA were enrolled in this study at 11 large German paediatric rheumatology units within the first 12 months after diagnosis. Laboratory and clinical parameters such as JIA core criteria and data on the medication used were collected every 3 months. Non-parametric statistical testing was performed for the comparison of the JIA core criteria at follow-up. Generalised linear models were used to analyse differences in the rates at which inactive disease was reached and to determine potential predictors. RESULTS: Of the 695 patients with JIA included in this analysis, approximately 75% experienced a period of inactive disease under treatment with disease-modifying antirheumatic drugs and systemic steroids in most cases with systemic-onset JIA or polyarthritis at least once during the first 12 months in ICON. Significant improvements were observed in all JIA core criteria, in disease activity and in functional status from baseline to the 12-month follow-up. Younger age at onset, a shorter duration between symptom onset and diagnosis and a positive antinuclear antibody status increased the probability of attaining an inactive disease state. CONCLUSIONS: The 12-month outcome of JIA was good under real-life conditions, with half of the patients having attained inactive disease with contemporary treatments. Since a short duration between symptom onset and diagnosis was correlated to a period of inactive disease, children suspected of having JIA should be transferred to specialised care as soon as possible.