Monitoring health-related quality of life (HRQoL) by using electronic patient-reported outcomes (ePROs) has been only minimally evaluated in pediatrics. Children with juvenile idiopathic arthritis ...(JIA) are at risk for HRQoL problems. The aim of this study was to investigate the effectiveness of ePROs in clinical pediatric rheumatology care.
All children (aged 0-18 years) with JIA visiting any of the 4 pediatric rheumatology clinics in Amsterdam between February 2009 and February 2010 were eligible for this sequential cohort intervention study. Before an outpatient consultation, children (aged 8-18 years) or parents (of children aged 0-7 years) completed web-based questionnaires. The resulting ePROfile was provided to the pediatric rheumatologist (PR). The study was divided into a control period in which the ePROfile was not discussed during consultation, and an intervention period in which the ePROfile was provided and discussed during consultation. Effectiveness was evaluated in terms of communication about different HRQoL topics, referral to a psychologist, and satisfaction with the consultations.
Out of the eligible JIA patients, 176 (65%) participated in the study. Use of the ePROfile increased discussion of psychosocial topics (P < .01), as well as the PR's satisfaction with provided care during consultation (P < .01). The use of ePROfiles did not affect referrals to a psychologist or parental satisfaction. Parents and PRs evaluated the use of the ePROfile as positive in 80% to 100% of the consultations.
Our web-based application to systemically monitor HRQoL problems in pediatric rheumatology contributed significantly to communication about psychosocial issues in a positive way. We recommend implementation of ePROs in pediatric clinical practice.
Objectives
To assess the diagnostic accuracy and reliability of MRI without contrast enhancement in the evaluation of JIA knee joint abnormalities.
Methods
JIA patients with clinically active knee ...involvement were prospectively studied using an 1-T open-bore magnet. MRI features were independently evaluated by two readers using the JAMRIS system. The first reading included unenhanced images, whereas complete image sets were available for the second reading.
Results
Imaging findings from 73 patients were analysed. Agreement between Gd-enhanced (+Gd) and Gd-unenhanced (−Gd) MRI scores of bone marrow changes, cartilage lesions and bone erosions was good concerning sensitivity, specificity, negative predictive value and positive predictive value. Inter-observer agreement was good for both −Gd and +Gd scores (ICC = 0.91–1.00, 0.93–1.00, respectively). Regarding the assessment of synovial hypertrophy, specificity of −Gd was high (0.97), but the sensitivity of unenhanced MRI was only 0.62. Inter-reader agreement for +Gd MRI was ICC = 0.94; however, omitting post-Gd acquisitions increased inter-reader variation (ICC = 0.86).
Conclusions
If Gd-enhanced MRI is the reference standard, omitting Gd contrast medium is irrelevant for the assessment of bone marrow changes, cartilage lesions and bone erosions as joint abnormalities in JIA. Omitting intravenous Gd in the MRI assessment of joints in JIA is inadvisable, because it decreases the reliability of detecting synovial disease.
Key Points
•
Magnetic resonance imaging is increasingly used to assess juvenile idiopathic arthritis.
•
Synovial hypertrophy, a marker of JIA activity, is well shown by MRI.
•
Omitting intravenous contrast medium decreases the reliability of synovial hypertrophy scores.
•
Bone marrow, cartilage and erosions can be reliably evaluated without contrast enhancement.
•
In the evaluation of JIA disease activity, unenhanced MRI is inadvisable.
Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (sJIA) are two distinct systemic inflammatory diseases of childhood. Each diagnosis is based on criteria, but numerous clinical ...features are overlapping. As no specific diagnostic tests are available, differentiation between both disease entities can be challenging. Here, we describe the disease course of patients with co-diagnosis of both KD and sJIA (KD/sJIA). All our KD (
= 1765) and sJIA (
= 112) cases were critically reviewed for co-diagnosis of KD/sJIA. Eight KD/sJIA cases were identified and their clinical presentation, treatment regimens, coronary artery outcome and complications are herein described. Each KD/sJIA patient fulfilled diagnostic criteria for KD and for sJIA. Ongoing fever, rash and arthritis were present in each patient. The KD/sJIA patients had recalcitrant KD requiring multiple doses of intravenous immunoglobulin and steroids. Five patients had coronary artery dilatation at KD diagnosis, which resolved in all by 6 weeks. Pericardial effusion was present in 5 patients. One KD/sJIA patient developed macrophage activation syndrome. In conclusion, a small proportion (0.5%) of our KD patients evolved into sJIA, and 7% of our sJIA population presented initially as KD. KD/sJIA patients were characterized by a recalcitrant KD course and a high prevalence of coronary artery dilatation. Patients with co-diagnoses may provide a clue to potentially shared immunopathology in KD and sJIA, leading us to posit that both entities may be part of the same clinical spectrum.
Objectives
To assess the reliability and responsiveness of a new Juvenile Arthritis MRI Scoring (JAMRIS) system for evaluating disease activity of the knee.
Methods
Twenty-five juvenile idiopathic ...arthritis (JIA) patients with clinical knee involvement were studied using open-bore 1-T MRI. MRI features of synovial hypertrophy, bone marrow changes, cartilage lesions and bone erosions were independently scored by five readers using the JAMRIS system. In addition, the JAMRIS system was determined to be a follow-up parameter by two readers to evaluate the response to therapy in 15 consecutive JIA patients.
Results
Inter-reader (ICCs 0.86–0.95) and intra-reader reliability (ICCs 0.92–1.00) for the scoring of JAMRIS features was good. Reliability of the actual scores and changes in scores over time was good for all items: ICCs 0.89–1.00, 0.87–1.00, respectively. Concerning therapy response, the mean synovial hypertrophy scores decreased significantly (mean 1.1 point;
P
< 0.001, SRM = −0.65). No change was observed with respect to bone marrow change, cartilage lesion and bone erosion scores.
Conclusions
The JAMRIS proved to be a simple and highly reliable assessment score in the evaluation of JIA disease activity of the knee. The JAMRIS system may serve as an objective and accurate outcome measure in future research and clinical trials.
Key Points
•
MRI is increasingly used to diagnose and assess juvenile idiopathic arthritis
.
•
A simple and reliable scoring method would help monitor progress and research
.
•
The Juvenile Arthritis MRI Scoring
(
JAMRIS
)
system provides reliable objective measures
.
•
JAMRIS evaluates synovial hypertrophy
,
bone marrow changes
,
cartilage lesions and bone erosions
.
•
The JAMRIS system can detect therapeutic response and should help future research
.
Abstract only Background: Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. We critically reviewed children with KD and sJIA to ...identify those with both diagnoses in order to characterize discriminating findings at baseline. Methods: Data from prospectively acquired KD (n=1765) and sJIA (n=112) cohorts were reviewed for common patients (1990-2011). Those with both diagnoses (KD/sJIA n=8) were reviewed for clinical presentation, laboratory investigations, treatment regimens, coronary artery outcome and complications including macrophage activation syndrome, and results were compared to the overall KD cohort. Results: All children with KD/sJIA fulfilled diagnostic criteria for KD and sJIA (ILAR classification). Co-diagnosis was present in 0.45% (8 of 1765) and 7.1% (8 of 112) of those with KD and sJIA, respectively. Time between diagnosis of KD and presumptive diagnosis of sJIA was a median of 24 days (IQR 21-45 days). KD/sJIA patients had bilateral conjunctival injection less frequently, lower hepatic transaminases together with signs of more intense inflammation as expressed a by higher white blood cell count and lower albumin than the KD cohort alone. All KD/sJIA patients had recalcitrant disease consisting of prolonged fever requiring multiple doses of intravenous immunoglobulin and steroids. Coronary artery abnormalities (CAAs) were observed in 5 KD/sJIA patients. Macrophage activation syndrome occurred in one KD/sJIA patient and in 0.9% and 8% of KD patients and sJIA patients respectively. Conclusions: A small portion of our patients with KD developed subsequent sJIA. KD/sJIA patients were characterized by more intense inflammation at initial presentation, a recalcitrant disease course and a high prevalence of CAAs. These patients may provide clues to potentially shared immunopathology. The clinical presentations of MAS, KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 3 entities are syndrome complexes defined by massive immune activation. We propose that the intensity and duration of the immune response may be the key distinguishing features, which dictate which one of these clinical syndromes the affected child presents with.
Objectives
To assess the value of magnetic resonance imaging (MRI) in discriminating between active and inactive juvenile idiopathic arthritis (JIA) patients and to compare physical examination ...outcomes with MRI outcomes in the assessment of disease status in JIA patients.
Methods
Consecutive JIA patients with knee involvement were prospectively studied using an open-bore MRI. Imaging findings from 146 JIA patients were analysed (59.6 % female; mean age, 12.9 years). Patients were classified as clinically active or inactive. MRI features were evaluated using the JAMRIS system, comprising validated scores for synovial hypertrophy, bone marrow oedema, cartilage lesions and bone erosions.
Results
Inter-reader reliability was good for all MRI features (intra-class correlation coefficient ICC = 0.87–0.94). No differences were found between the two groups regarding MRI scores of bone marrow oedema, cartilage lesions or bone erosions. Synovial hypertrophy scores differed significantly between groups (
P
= 0.016). Nonetheless, synovial hypertrophy was also present in 14 JIA patients (35.9 %) with clinically inactive disease. Of JIA patients considered clinically active, 48.6 % showed no signs of MRI-based synovitis.
Conclusions
MRI can discriminate between clinically active and inactive JIA patients. However, physical examination is neither very sensitive nor specific in evaluating JIA disease activity compared with MRI. Subclinical synovitis was present in >35 % of presumed clinically inactive patients.
Key points
•
MRI is sensitive for evaluating juvenile idiopathic arthritis
(
JIA
)
disease activity
.
•
Contrast
-
enhanced MRI can distinguish clinically active and inactive JIA patients
.
•
Subclinical synovitis is present in 35
.
9
%
of presumed clinically inactive patients
.
•
Physical examination is neither sensitive nor specific in evaluating JIA disease activity
.
High-resolution peripheral quantitative computed tomography (HR-pQCT) has the potential to improve radiographic progression determination in clinical trials and longitudinal observational studies. ...The goal of this work was to describe the current state of research presented at Outcome Measures in Rheumatology (OMERACT) 2016 and ensuing future directions outlined during discussion among attendees.
At OMERACT 2016, SPECTRA (Study grouP for xtrEme-Computed Tomography in Rheumatoid Arthritis) introduced efforts to (1) validate the HR-pQCT according to OMERACT guidelines, focusing on rheumatoid arthritis (RA), and (2) find alternatives for automated joint space width (JSW) analysis. The Special Interest Group (SIG) was presented to patient research partners, physicians/researchers, and SIG leaders followed by a 40-min discussion on future directions.
A consensus definition for RA erosion using HR-pQCT was demonstrated through a systematic literature review and a Delphi exercise. Histopathology and perfusion studies were presented that analyzed the true characteristics of cortical breaks in HR-pQCT images, and to provide criterion validity. Results indicate that readers were able to discriminate between erosion and small vascular channels. Moderate reliability (ICC 0.206-0.871) of direct erosion size measures was shown, which improved (> 0.9) only when experienced readers were considered. Quantification of erosion size was presented for scoring, direct measurement, and volumetric approaches, as well as a reliability exercise for direct measurement. Three methods for JSW measurement were compared, all indicating excellent reproducibility with differences at the extremes (i.e., near-zero and joint edge thickness).
Initial reports on HR-pQCT are promising; however, to consider its use in clinical trials and longitudinal observational studies, it is imperative to assess the responsiveness of erosion measurement quantification.
To evaluate whether clinical disease activity findings during 1-year followup of patients with juvenile idiopathic arthritis (JIA) is associated with changes of magnetic resonance imaging (MRI)-based ...disease activity scores.
Patients with JIA who had active knee involvement were studied using an open-bore MRI. After followup of a median of 1.3 years, patients were re-evaluated and classified as improved or unimproved according to the American College of Rheumatology Pediatric-50 (ACR-Ped50) criteria. Baseline and followup MRI features were scored by 2 readers using the Juvenile Arthritis MRI Scoring (JAMRIS) system, comprising validated scores for synovial hypertrophy, bone marrow changes, cartilage lesions, and bone erosions.
Data of 40 patients were analyzed (62.5% female, mean age 12.2 yrs). After followup, 27 patients (67.5%) were classified as clinically improved, whereas 13 patients (32.5%) showed no clinical improvement. The clinically improved patients showed a significant reduction in synovial hypertrophy scores during followup (p < 0.001), with substantial effects (standardized response mean -0.70). No such changes were observed for any of the other MRI features. Significant differences were detected regarding a change in synovial hypertrophy scores comparing clinically improved and unimproved patients (p = 0.004), without statistically significant differences for changes in scores for bone marrow changes (p = 0.079), cartilage lesions (p = 0.165), and bone erosions (p = 0.078).
This is one of the first studies to provide evidence for MRI-based improvement upon followup in JIA patients with knee involvement. There is a strong association with clinical improvement according to the ACR-Ped50 criteria and changes in MRI-based synovial hypertrophy scores, supporting the role of MRI as a responsive outcome measure to evaluate disease activity with antiinflammatory treatment strategies.
This paper aims to evaluate disease course and outcome of patients in the first 2 years after diagnosis of juvenile idiopathic arthritis (JIA) when treated according to local standard of clinical ...care, focusing on achievement of inactive disease, functional ability and radiological joint damage.
A retrospective inception cohort study of children with JIA, diagnosed between January 2003 and June 2007 and treated in referral centres in Amsterdam, was carried out. Disease status was determined for every outpatient-clinic visit. Data regarding medication, functional outcome and radiography were recorded.
One hundred and forty-nine consecutive newly diagnosed JIA patients were included. Median age at diagnosis was 11.8 years; median follow-up was 33 months. Synthetic DMARDs (sDMARDs) were used by 95% of patients, including methotrexate in 85%, sulfasalazine in 41% and biologics in 20%. sDMARDs were started within median 1 month after diagnosis. During follow-up, 77% of patients achieved a total of 244 episodes of inactive disease (ID). ID was reached after median 10 months. No baseline predictive factors for achievement of ID could be identified. After 2 years a median CHAQ score of 0.6 was reported. Radiological joint damage occurred at some point in 18 patients (12%); 10 of these patients developed erosions within median 20 months after their first clinic visit.
With current management strategies in daily clinical practice, 77% of newly diagnosed JIA patients achieved a first episode of inactive disease within a median of 10 months. After 2 years, patients reported moderate functional disability and more than 10% showed radiological evidence of joint damage.
Background
MRI is the most sensitive imaging modality in juvenile idiopathic arthritis (JIA), but has practical limitations. Optimizing the scanning protocol is, therefore, necessary to increase ...feasibility and patient comfort.
Objective
To determine the feasibility of bilateral non-contrast-enhanced open-bore MRI of knees and to assess the presence of literature-based MRI features in unsedated children with JIA.
Materials and methods
Children were classified into two clinical subgroups: active arthritis (group 1;
n
= 29) and inactive disease (group 2;
n
= 18). MRI features were evaluated using a literature-based score, comprising synovial hypertrophy, cartilage lesions, bone erosions, bone marrow changes, infrapatellar fat pad heterogeneity, effusion, tendinopathy and popliteal lymphadenopathy.
Results
The MRI examination was successfully completed in all 47 children. No scan was excluded due to poor image quality. Synovial hypertrophy was more frequent in group 1 (36.2%), but was also seen in 19.4% of the knees in group 2. Infrapatellar fat pad heterogeneity was more prevalent in group 2 (86.1%;
P
= 0.008). Reproducibility of the score was good (Cohen kappa, 0.49–0.96).
Conclusion
Bilateral non-contrast-enhanced open-bore knee MRI is feasible in the assessment of disease activity in unsedated children with JIA. Signs differing among chidren with active and inactive disease include infrapatellar fat pad heterogeneity and synovial hypertrophy.
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