Patient perceived needs and experiences of person-centered care in patients with inflammatory arthritis.

BACKGROUND: Health care is shifting toward a person-centered care (PCC) approach. For implementation of PCC, there may be a special role for nurse practitioners (NPs). PURPOSE: The aim of this study was to explore the patient-perceived levels of and needs for of PCC in inflammatory arthritis patients who visited the NP at the outpatient clinic of an academic hospital in the Netherlands. METHODS: A cross-sectional study was performed. Disease characteristics were inventoried from the patient records. Patients filled out the PCCoc/rheum instrument, an instrument to measure patient perceived PCC, and a questionnaire based on the 14 life areas of the Self-Management Web, extended with areas including pain, fatigue, and night's rest. Participants were asked which life areas caused problems, and whether these problems were discussed. Mean values were calculated for normally distributed data and medians for nonnormally distributed data. RESULTS: Most of the patients had well-controlled disease (86.1%). The mean score of the PCCoc/rheum was 55.3 (SD 8.1). Patients experienced most problems in life areas fatigue (37.3%) and pain (35.3%), these were also the life areas that were most often addressed at consultation. The life areas that gave problems and that were least addressed during consultation were intimate relationships & sexuality (66.7%) and household chores (58.8%). CONCLUSIONS: Despite an overall high level of patient perceived PCC delivered by NPs, patient with low disease activity frequently reported problems in life areas not addressed at consultation. IMPLICATIONS FOR PRACTICE: Implementation of the Self-Management Web and changing the focus of NP consultations may help to improve accommodating individual patient needs.

Human TBK1 deficiency leads to autoinflammation driven by TNF-induced cell death.

TANK binding kinase 1 (TBK1) regulates IFN-I, NF-κB, and TNF-induced RIPK1-dependent cell death (RCD). In mice, biallelic loss of TBK1 is embryonically lethal. We discovered four humans, ages 32, 26, 7, and 8 from three unrelated consanguineous families with homozygous loss-of-function mutations in TBK1. All four patients suffer from chronic and systemic autoinflammation, but not severe viral infections. We demonstrate that TBK1 loss results in hypomorphic but sufficient IFN-I induction via RIG-I/MDA5, while the system retains near intact IL-6 induction through NF-κB. Autoinflammation is driven by TNF-induced RCD as patient-derived fibroblasts experienced higher rates of necroptosis in vitro, and CC3 was elevated in peripheral blood ex vivo. Treatment with anti-TNF dampened the baseline circulating inflammatory profile and ameliorated the clinical condition in vivo. These findings highlight the plasticity of the IFN-I response and underscore a cardinal role for TBK1 in the regulation of RCD.

Successful implementation of a clinical transition pathway for adolescents with juvenile-onset rheumatic and musculoskeletal diseases.

BACKGROUND: In 2008 a clinical transition pathway for young people with juvenile-onset rheumatic and musculoskeletal diseases (jRMD) aiming at improving transitional care was instituted. Historical data on drop-out rate in our clinic was 35%, one year before the implementation of the transition pathway. This study aims to I) evaluate the effectiveness of the clinical transition pathway, II) evaluate the experiences and satisfaction of YP with the transitional process and evaluate their perceived self-management skills. METHODS: Young people with any jRMD transferred from the pediatric to the adult rheumatology department in our academic center were eligible to enroll in this quantitative cross-sectional observational study between 2009 and 2015. Notably in 2012, we created a dedicated adolescent JIA-clinic, located at the adult rheumatology department. Electronic patient records from all young people that were transferred between 2009 and 2015 were reviewed for drop-out of care. Young people were asked to rate a VAS for 'satisfaction with transition' and to complete the "on your own feet transfer experience scale" (OYOF-TES)-questionnaire regarding their experiences and satisfaction with transition. Self-management skills were measured with the "on your own feet self-efficacy scale" (OYOF-SES)-questionnaire. RESULTS: One hundred fifty-four young people were transferred to the adult department, of which 76 were transferred to the dedicated adolescent JIA-clinic. The mean age at transfer was 17.8 years for YP transferred to the adult clinic and 15.2 years for transfer to the adolescent clinic. Drop-out of care rate one year after transfer was 5.1% in the adult clinic and 1.3% in the adolescent JIA-clinic. Response rate of the returned questionnaires was 61% for the adolescent JIA clinic and 36% for the adult clinic. There was no difference between responders and non-responders in demographics and disease type besides age (non-responders were significantly younger). Young people transferred to the adult and adolescent JIA-clinic both had high scores on the satisfaction scale (7.7 and 7.5 on the VAS-scale and 72.0 and 74.5 on the OYOF-TES). Self-efficacy scores were high for both groups, with OYOF-SES 59.7 for those transferred to the adult clinic and 58.2 for those transferred to the adolescent JIA-clinic. CONCLUSION: The implementation of the clinical transition pathway has led to a substantial improvement of patient care during the transitional process leading to low drop-out of care rate and high scores on satisfaction with transition. High scores on the self-reported self-efficacy scale suggests confidence of young people to have achieved sufficient skills to successfully manage their disease.

The Dutch version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Dutch language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 209 JIA patients (14.3% systemic, 39.7% oligoarticular, 25.8% RF negative polyarthritis, 20.2% other categories) and 107 healthy children were enrolled in two centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Dutch version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Disease activity and dropout in young persons with juvenile idiopathic arthritis in transition of care: a longitudinal observational study.

OBJECTIVES: Reaching a certain age, juvenile idiopathic arthritis (JIA) patients in paediatric care are transferred to adult care. An increased disease activity after transfer and increased dropout has been suggested, however, evidence is scarce. Our aim is to determine whether the process of transition is associated with increased disease-activity and dropout, and to identify associated factors. METHODS: During a 3-year prospective transition cohort study, paediatric patients (14-17yrs) were transferred to adult care. Paediatric (10-13yrs) and adult JIA patients (18-27yrs) were used as control groups. Demographic and disease-related items were obtained yearly. Non-parametric tests were used to compare differences between the groups and mixed models to evaluate disease activity over time, measured by JADAS27 and DAS28. Dropout was defined as not attending the clinic for 2 consecutive visits. RESULTS: Groups did not differ regarding baseline variables of subtype, gender, uveitis, ANA-, RF- or HLA B27-positivity and current or past DMARD use. Median disease activity was not different between groups during follow-up. Transfer was not associated with disease activity. Dropout rate was 12%, and was significantly higher in patients under transition (22%) compared with paediatric (3%) and adult care (10%). Patients who dropped out had significantly lower disease activity at baseline and were using less MTX, but did not differ regarding subtype, ANA, RF and HLA-B27. CONCLUSIONS: The process of transition in JIA is not associated with an increase in disease activity, however, this period carries a risk for drop out especially in patients with low disease activity.

Development of a clinical transition pathway for adolescents in the Netherlands.

AIMS: To explore how young people with juvenile-onset rheumatic and musculoskeletal diseases (jRMDs) and their parent(s) experience care during preparation for the upcoming transfer to adult services, and to develop a clinical transition pathway. METHOD: A survey was conducted with 32 young people aged between 14 and 20 years with jRMDs, and their parents ( n =33), treated at the department of paediatric rheumatology in a tertiary care children's hospital in the Netherlands. RESULTS: More than 30% of young people would have liked to discuss topics such as educational and vocational choices in a clinic, but did not get the opportunity. Preparation for transition was poor as was training in self-management skills. One third of parents had feelings of anxiety about the upcoming transfer. Results from the survey and evidence-based principles of transitional care were used to develop the clinical transition pathway. The pathway focuses on starting transition early, developing self-management skills, joint consultations and supporting parents in giving young people control of their clinical care. CONCLUSION: Care does not currently meet the needs of young people in the process of transition to adult rheumatology services. The clinical transition pathway developed as a result of the study is a tool that may improve this process.

Short term effectiveness and experiences of a peer guided web-based self-management intervention for young adults with juvenile idiopathic arthritis.

BACKGROUND: A web-based self-management intervention guided by peer-trainers was developed to support young adults' self-management in coping with Juvenile Idiopathic Arthritis (JIA). To investigate its effectiveness, a randomized controlled trial (RCT) was conducted. In addition, the content of the chat and participants' goals were studied to identify underlying processes. METHODS: An RCT with a six-month follow up period was conducted among 72 young adults with JIA, aged between 16 and 25 years old, randomly assigned to the intervention or to the usual care control group. After 24 weeks, in both groups 24 participants completed all measurements. Intentions to treat analyses were carried out by means of linear mixed models for longitudinal measurements. With self-efficacy as primary outcome, self-management, disease activity, quality of life, absenteeism of school/work, health care medication use and adherence to the intervention were studied. The participants' goals, personal achievements, interactions on the chat, and their appreciation of the intervention were analyzed using thematic analyses. RESULTS: No significant differences were found on self-efficacy, quality of life, and self-management between the participants of the control group and the intervention group. In the intervention group, modeling and sharing experiences were the most recognized themes. Fifty-five goals were formulated and divided into the following categories: improvement and maintaining balance, setting and recognizing boundaries, communicating and coping with incomprehension. Adherence, appreciation of the own learning experience, and personal achievements were rated positively. CONCLUSION: The web-based intervention did not lead to an improvement of self-efficacy. However, additional qualitative analyses showed that the intervention was appreciated and valuable for the participants. More research is needed on how to measure the added value of this intervention compared to the usual care. TRIAL REGISTRATION: Trial registration number NTR4679 .

EULAR/PReS standards and recommendations for the transitional care of young people with juvenile-onset rheumatic diseases.

To develop standards and recommendations for transitional care for young people (YP) with juvenile-onset rheumatic and musculoskeletal diseases (jRMD). The consensus process involved the following: (1) establishing an international expert panel to include patients and representatives from multidisciplinary teams in adult and paediatric rheumatology; (2) a systematic review of published models of transitional care in jRMDs, potential standards and recommendations, strategies for implementation and tools to evaluate services and outcomes; (3) setting the framework, developing the process map and generating a first draft of standards and recommendations; (4) further iteration of recommendations; (5) establishing consensus recommendations with Delphi methodology and (6) establishing standards and quality indicators. The final consensus derived 12 specific recommendations for YP with jRMD focused on transitional care. These included: high-quality, multidisciplinary care starting in early adolescence; the integral role of a transition co-ordinator; transition policies and protocols; efficient communications; transfer documentation; an open electronic-based platform to access resources; appropriate training for paediatric and adult healthcare teams; secure funding to continue treatments and services into adult rheumatology and the need for increased evidence to inform best practice. These consensus-based recommendations inform strategies to reach optimal outcomes in transitional care for YP with jRMD based on available evidence and expert opinion. They need to be implemented in the context of individual countries, healthcare systems and regulatory frameworks.

Use and perceived relevance of health-related Internet sites and online contact with peers among young people with juvenile idiopathic arthritis.

OBJECTIVE: To evaluate the frequency of use and relevance of health-related Internet (HRI) sites and online peer support groups and their association with demographic, disease-related and psychosocial variables in young people with JIA. METHODS: In a cross-sectional study, 176 young people (10-27 years of age) with JIA were asked to complete a questionnaire. The frequency of using HRI sites (regarding information, medication use and aspects of JIA relating to social life), online peer contact and perceived relevance of HRI sites and online peer communication were determined. Associations with demographic variables, disease activity, medication, emotional behaviour and coping were also examined. RESULTS: Seventy-one per cent of the 142 respondents had used the Internet to search for general information on JIA, but specific topics, such as medication, were searched for less often. Twenty-five per cent of respondents had visited a forum or had contacted peers online. The perceived relevance of HRI sites and online peer contact was rated low (median 2.0 and 1.0, respectively; scale 0-10). Apart from female gender (P < 0.01), none of the demographic and disease-related factors were associated with HRI site use. Coping styles, confrontation and reassuring thoughts were associated with increased HRI site use, but only in males. Internalizing and externalizing problem behaviour were not significantly associated. CONCLUSION: The frequency of HRI site use among young people with JIA was less than expected and was considered of low relevance. HRI sites in their present form cannot replace traditional information as an additional source to increase knowledge.

Patient-reported joint count in juvenile idiopathic arthritis: the reliability of a manikin format.

OBJECTIVE: To evaluate the reliability of a manikin format, patient-reported joint count in juvenile idiopathic arthritis (JIA), and to detect changes in agreement at a second visit. METHODS: Patients with JIA aged 12-21 were asked to mark joints with active arthritis on a manikin before their regular clinic visit. The physician then performed a joint count without having seen the patient's assessment. Agreement between scores of physician-reported and patient-reported joint counts was assessed using ICC. Kappa statistics were used to assess reliability of scoring individual joints. RESULTS: The study included 75 patients with JIA. In general, patients had a low number of active joints (median 1 joint, indicated by the physician). ICC was moderate (0.61) and κ ranged from 0.3-0.7. At the second visit, κ were similar; the ICC was 0.19. When a patient scored 0 joints, the physician confirmed this 93%-100% of the time. When the patient marked ≥ 1 joints, the physician confirmed arthritis 59%-76% of the time. Sensitivity to change was moderate. CONCLUSION: Agreement between physician and patient on the number of joints with active arthritis was reasonable. Untrained patients tended to overestimate the presence of arthritis when they marked active joints on a manikin-format joint count. When the patient indicated absence of arthritis, the physician usually confirmed this. As the agreement did not improve at followup, future research should focus on the possibility of achieving this through training. For now, the patient-reported joint count cannot replace the physicians' joint count in clinical practice; it may be used in epidemiological studies with caution.

Trends in prescription of biological agents and outcomes of juvenile idiopathic arthritis: results of the Dutch national Arthritis and Biologics in Children Register.

BACKGROUND: Treatment of juvenile idiopathic arthritis (JIA) has changed dramatically since the introduction of biological agents in 1999. OBJECTIVE: To evaluate trends in prescription patterns of biological agents and the subsequent outcome of JIA. METHODS: The Arthritis and Biologics in Children register (multicentre prospective observational study) aimed to include all consecutive patients with JIA in the Netherlands who had started biological agents since 1999. Patients were divided according to year of introduction of first biological agent. Patient characteristics at introduction of the first biological agent and its effectiveness were analysed over 12 years. RESULTS: 335 patients with non-systemic JIA and 86 patients with systemic JIA started a biological agent between 1999 and 2010. Etanercept remained the most often prescribed biological agent for non-systemic JIA; anakinra became first choice for systemic JIA. The use of systemic glucocorticoids and synthetic disease-modifying antirheumatic drugs before biological agents decreased. During these 12 years of observation, biological agents were prescribed earlier in the disease course and to patients with lower baseline JADAS (Juvenile Arthritis Disease Activity Score) disease activity. All baseline disease activity parameters were lowered in patients with non-systemic JIA. In systemic JIA, prescription patterns changed towards very early introduction of biological agents (median 0.4 years of disease duration) in patients with a low number of joints with active arthritis and high erythrocyte sedimentation rates. These changes for both systemic and non-systemic JIA resulted in more patients with inactive disease after 3 and 15 months of treatment. CONCLUSIONS: Biological agents are increasingly prescribed, earlier in the disease and in patients with JIA with lower disease activity. These changes are accompanied by better short-term disease outcomes.

Treatment choices of paediatric rheumatologists for juvenile idiopathic arthritis: etanercept or adalimumab?

OBJECTIVES: To evaluate differences in baseline characteristics between etanercept- and adalimumab-treated JIA patients and to reveal factors that influence the choice between these TNF inhibitors, which are considered equally effective in the recent ACR recommendations for JIA treatment. METHODS: Biologic-naïve JIA patients with active arthritis who started treatment with adalimumab or etanercept between March 2008 and December 2011 were selected from the Dutch Arthritis and Biologicals in Children register. Baseline characteristics were compared. Focus group interviews with paediatric rheumatologists were performed to evaluate factors determining treatment choices. RESULTS: A total of 193 patients started treatment with etanercept and 21 with adalimumab. Adalimumab-treated patients had longer disease duration prior to the start of biologics (median 5.7 vs 2.0 years) and more often a history of uveitis (71% vs 4%). Etanercept-treated patients had more disability at baseline (median Childhood Health Assessment Questionnaire score 1.1 vs 0.4) and more active arthritis (median number of active joints 6 vs 4). The presence of uveitis was the most important factor directing the choice towards adalimumab. Factors specific for the paediatric population-such as painful adalimumab injections-as well as the physician's familiarity with the drug accounted for the preference for etanercept. CONCLUSION: Although the two TNF inhibitors are considered equally effective, in daily practice etanercept is most often prescribed; adalimumab is mainly preferred when uveitis is present. In choosing the most suitable biologic treatment, paediatric rheumatologists take into account drug and patient factors, considering newly published data and cautiously implementing this into daily care.

Tumour necrosis factor-blocking agents in persistent oligoarticular juvenile idiopathic arthritis: results from the Dutch Arthritis and Biologicals in Children Register.

OBJECTIVE: Because TNF inhibitors are not approved for persistent oligoarticular JIA (oJIA), although they are used off-label, we evaluated their effectiveness in patients in this category. METHODS: Persistent oJIA patients were selected from the Dutch Arthritis and Biologicals in Children (ABC) register, an ongoing multicentre prospective study that aims to include all Dutch children with JIA using biologic agents. Response was assessed by the JIA core-set disease activity variables and modified Wallace criteria for inactive disease. RESULTS: Until February 2011, 16 persistent oJIA patients (68.8% females) had been included in the register. Median age of onset was 8.4 years [interquartile range (IQR) 2.1-13.5 years]; history of uveitis in 18.8%; ANA-positive 56.3%. All had previously used MTX, and 81.3% had used IA CSs. Median follow-up after the introduction of biologic treatment was 13.7 months (IQR 8.3-16.7 months). Fourteen patients started etanercept and two patients who had active arthritis as well as uveitis started adalimumab. Although patients with persistent oJIA had few affected joints [median of two active joints at the start of biologic (IQR 1-3)], the patient/parent assessments of pain [median visual analogue score (VAS) 51 (IQR 1-64)] and well-being [median VAS 44 (IQR 6-66)] were high. Additionally, their physician evaluated the disease activity as moderately high [median VAS 36 (IQR 4-65)]. After 3 months this decreased to 0 (IQR 0-30) and 63% achieved inactive disease. After 15 months the disease was inactive in 9/10 observed patients. TNF inhibitors were tolerated well. CONCLUSION: TNF blocking agents seem an effective and justifiable option in persistent oJIA when treatment with IA CS injections and MTX has failed.

Effectiveness and safety of a second and third biological agent after failing etanercept in juvenile idiopathic arthritis: results from the Dutch National ABC Register.

OBJECTIVE: To evaluate the effectiveness and safety of switching to a second or third biological agent in juvenile idiopathic arthritis (JIA) after etanercept failure. METHODS: The Arthritis and Biologicals in Children Register aims to include all Dutch JIA patients who have used biological agents. Data on the disease course were used to estimate drug survival with Kaplan-Meier and calculate adverse event (AE) rates. RESULTS: Of 307 biologically naive JIA patients who started etanercept, 80 (26%) switched to a second and 22 (7%) to a third biological agent. During 1030 patient-years of follow-up after the introduction of etanercept, 49 switches to adalimumab, 28 infliximab, 17 anakinra, four abatacept and four trial drugs were evaluated. 84% (95% CI 80% to 88%) of patients who started etanercept as a first biological agent were, after 12 months, still on the drug, compared with 47% (95% CI 35% to 60%) who started a second and 51% (95% CI 26% to 76%) who started a third biological agent. Patients who switched because of primary ineffectiveness continued the second agent less often (32%, 95% CI 12% to 53%). After etanercept failure, drug continuation of adalimumab was similar to infliximab for patients with non-systemic JIA; anakinra was superior to a second TNF-blocker for systemic JIA. AE rates within first 12 months after initiation were comparable for each course and each biological agent. CONCLUSIONS: Switching to another biological agent is common, especially for systemic JIA patients. A second (and third) agent was less effective than the first. The choice of second biological agent by the physician mainly depends on availability and JIA category.

Aerobic capacity and disease activity in children, adolescents and young adults with juvenile idiopathic arthritis (JIA).

BACKGROUND: As patients with juvenile idiopathic arthritis (JIA) progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovascular morbidity and mortality in late adulthood. The objectives of the current study are to examine the aerobic capacity and its relation to parameters of disease activity in children, adolescents and young adults with JIA. METHODS: Sixty-three patients with JIA (aged 10-27 years) were cross sectional studied regarding their aerobic capacity and correlations were made to demographic, disease-related variables, and medication utilization. in a cross-sectional study group of 63 patients of all subtypes. Patients were divided in three age groups, 10-13 years; 14-17 years and 18-27 years. RESULTS: Reduced aerobic capacity is found in clinical remission as well as active disease in all subtypes and all age groups. Aerobic capacity is more impaired in active disease shown by DAS 28, JADAS 27, ESR and serum thrombocyte counts. Lower haemoglobin has a negative impact. Long-term used medication including methotrexate and corticosteroids didn't influence outcome. There is no association with current sports participation. CONCLUSION: Reduced aerobic capacity is present in adolescents and young adults with JIA, both in active disease and in patients with remission. Measures of aerobic capacity may serve as important outcome measure in JIA.

Evaluating score distributions in the revised Dutch version of the Childhood Health Assessment Questionnaire.

OBJECTIVES: Evaluating the original, and the revised version of the Dutch Childhood Health Assessment Questionnaire (CHAQ). To explore the effect of different score calculation methods and eight more challenging items as proposed by Lam et al. (2004) on the score distribution in a population of patients with Juvenile Idiopathic Arthritis (JIA). METHODS: Two convenience samples of 59 and 31 children with JIA were studied. Box-and-whisker plots and the Kolmogorov-Smirnov (K-S) one-sample test of normality were used, to explore the score distributions. RESULTS: The results of this study confirm a ceiling effect when using the original CHAQ-30 with either score calculation method. The original CHAQ with the added eight more challenging items and the "mean" score calculation method, as well as the revised CHAQ showed less ceiling effect. CONCLUSION: The original CHAQ-38 with the "mean" score calculation method as well as the revised CHAQ are a possible alternative for future studies. However, there is a need for further prospective studies to improve the CHAQ and to support our findings.