Juvenile Idiopathic Arthritis Activity Research Articles

Persistent improper upregulation of Th17 and TReg cells in patients with juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease. A break in the balance between Th17 and TReg cells has been reported as an important factor in the development of autoimmune diseases. This study aimed to analyze peripheral Th17 and TReg cell levels in patients with JIA. The balance of Th17 and TReg cells among active and inactive JIA patients and normal control subjects were compared. Human peripheral blood mononuclear cells were isolated from the patients and controls. Surface and intracellular staining for CD4, CD25, Foxp3, IL-17, Th17, and TReg were analyzed. Twenty-eight JIA patients, including 12 with active JIA and 16 with inactive JIA, and 20 health controls were analyzed. Patients with active JIA had higher Th17 (1.85±1.15 vs. 1.05±0.72, p = 0.008) and TReg cells (1.1±0.8 vs. 0.6±0.7, p = 0.04) levels than those with inactive JIA. Among active JIA patients, remission days were highly correlated with the CD4(+)IL17A(+) T cell percentage, 276.5 ± 137.40 days (range, 130 ∼ 525 days), p < 0.01. There were no differences in Th17/TReg percentage between JIA patients and controls in the peripheral blood. Th17 and TReg cell levels are elevated in patients with active JIA and there is no Th17/TReg imbalance. The higher Th17 level predicted longer period to reach disease inactive stage. Improper Th17 up-regulation might contribute to JIA activation.

Determinants of vitamin D levels in children, adolescents, and young adults with juvenile idiopathic arthritis.

Deficiency of 25-hydroxyvitamin D [25(OH)D] is reported to be common in patients with rheumatoid arthritis (RA); data in patients with juvenile idiopathic arthritis (JIA) are inconsistent. We assessed serum 25(OH)D in children, adolescents and young adults with JIA, in order to identify the risk factors for vitamin D deficiency in patients with JIA. We evaluated 152 patients with JIA: 115 female, 37 male, mean age 16.2 ± 7.4 yrs; evaluated by onset type, 96 had oligoarticular, 35 polyarticular, 7 systemic, and 14 enthesitis-related arthritis (ERA). Patients were compared with a control group matched for sex and age. All patients and controls underwent laboratory tests of plasma 25(OH)D, parathyroid hormone (PTH), calcium, phosphorus, and bone alkaline phosphatase levels, and dual-energy x-ray absorptiometry examination. Patients with JIA showed significantly reduced 25(OH)D levels compared to controls (p < 0.001), even divided into subtypes (oligoarticular, p < 0.05; polyarticular, p < 0.005; systemic, p < 0.001; ERA, p < 0.005). Patients with active disease and/or frequent relapses had significantly reduced 25(OH)D levels compared to patients with no active disease and no frequent flares (p < 0.005, respectively). Nevertheless, JIA patients had significantly higher PTH levels compared to controls (p < 0.0001). JIA patients with 25(OH)D deficiency showed a significantly lower bone mineral apparent density than those with normal 25(OH)D levels (p < 0.001). JIA patients have reduced 25(OH)D and higher PTH values. This may explain at least in part why JIA patients, despite more effective current drugs, do not achieve bone-normal condition over time. JIA patients with more severe disease could require higher supplementation of vitamin D to maintain normal 25(OH)D serum levels. Longterm studies are needed to investigate the relationship between serum 25(OH)D levels and disease activity in JIA.

AB0908 Evaluation of the Activity of Juvenile Idiopathic Arthritis by Jadas: Jadas ESR versus Jadas3

BackgroundThe Jadas (Juvenile arthritis Disease Activity Score) is a recently developed and validated for the assessment of the activity of juvenile idiopathic arthritis (JIA), including articular index composite score, physician...

AB0216 Serum Angiogenesis Markers and their Association with Ultrasound-Detected Synovitis in the Evaluation of Disease Activity in Juvenile Idiopathic Arthritis

BackgroundSynovial angiogenesis, defined as a complex process of developing new blood vessels from the existing microvascular bed, is considered to be an important early step in the course and pathogenesis...

AB0883 The Factors Affecting the FAT Body Mass in Patients with Juvenile Idiopathic Arthritis

ObjectivesTo determine the factors influencing the fat body mass in patients with juvenile idiopathic arthritis.MethodsA cross sectional study in patients with juvenile idiopathic arthritis (JIA) according to the International League...

AB0886 Bone Health Assessment of Patients with Juvenile Idiopathic Arthritis: A Comparison between DXA and Bonexpert

BackgroundChronic inflammatory activity in juvenile idiopathic arthritis (JIA) affects the bone, possibly resulting in growth abnormalities and decreased bone mineral density (BMD). Monitoring the bone status of JIA patients is...

AB0909 The Jadas in Assessing the Activity of Juvenile Idiopathic Arthritis: Jadas ESR versus Crp

BackgroundThe Jadas (Juvenile arthritis Disease Activity Score) is a recently developed and validated for the assessment of the activity of juvenile idiopathic arthritis (JIA) composite score it includes 4items. Articular...

A30: Assessment of Construct and Discriminative Validity of the 3‐Variable JADAS in Relation of Parent‐Reported Outcomes

Background/Purpose:An established approach to the measurement of disease activity in juvenile idiopathic arthritis (JIA) is based on the Juvenile Arthritis Disease Activity Score (JADAS). The feasibility of the JADAS for use in standard clinical practice might be enhanced by implementing of a 3‐variable version, which does not include the acute phase reactant. Recently, the 3‐variable JADAS was found to correlate closely with the original tool. However, the validity of this simplified version of the JADAS in the clinical setting is still insufficiently documented. Aim of the study was to further validate the 3‐variable version of the JADAS, hence named clinical JADAS (cJADAS), by assessing its relationship with parent‐reported outcomes (excluding the parent's rating of overall well‐being, which is included in the JADAS).Methods:The study was conducted in a cohort of 175 newly‐diagnosed consecutive JIA patients, first seen between 2007–2011 and then followed for 2 years. Clinical assessments were made at baseline and then at 6, 12, 18 and 24 months. At each visit, all parent reported outcomes included in the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) were assessed by a parent of each patient. Construct validity of the cJADAS was computed by calculating its Spearman's correlation with pain visual analog scale (VAS) and disease activity VAS. Discriminative validity was evaluated, by means of the non‐parametric analysis of variance (Kruskal‐Wallis test) or Mann‐Whitney U test, by examining the ability of cJADAS to discriminate between: 1) different categories of disease state (remission, persistent activity or flare), rated subjectively by the parents; 2) different categories of disease course (improved, stable or worsened), rated subjectively by the parents; 3) visit in which parents declared themselves as satisfied or not satisfied with the outcome of the illness; 4) different duration of morning stiffness.Results:The cJADAS was strongly correlated with pain VAS (r=0.71) and disease activity VAS (r=0.77). The cJADAS was significantly lower in patients rated as being in remission than in patients rated as being in persistent activity or flare (p &lt; 0.0001), in patients rated as improved or stable than in patients rated as worsened (p &lt; 0001), and in patients whose parents were satisfied with illness outcome than in those who were not (p &lt; 0.0001). The cJADAS was proportionally greater in parallel with the progressive increase in morning stiffness (p &lt; 0001).Conclusion:The cJADAS was strongly associated with all parent‐reported outcomes, which indicates that it reflects well the parent perception of the child's disease status and course.

A21: Physical Activity in Children with Juvenile Idiopathic Arthritis (JIA): The LEAP (Linking Exercise, Activity, and Pathophysiology in Childhood Arthritis) Study

Background/Purpose:Although children with JIA have lower fitness levels than healthy peers, little is known about their level of habitual physical activity. The LEAP study is a prospective longitudinal multicentre study of children and teens with JIA, aimed at describing the trajectory of physical activity (PA) in JIA, and its relationship to disease factors, inflammation, quality of life, bone health and muscle function. We report PA levels of children with JIA in early and late disease at study entry.Methods:We enrolled patients with JIA (aged 8–16 y) at 12 pediatric rheumatology centres in Canada as either an inception cohort (early disease; within 6 mos of diagnosis) or an established disease cohort (late disease; &gt; 2yr after diagnosis). We assessed PA with a validated Physical Activity Questionnaire for children (PAQ‐C, age 8–13 y) or teens (PAQ‐A, age 14–16 y). This 7‐day recall self‐report tool has scores from 0 (no PA)‐ 5(high PA). Participants record weekly PA across a wide range of activities and sports. Patients completed a pain scale (VAS 0–100), the Childhood Health Assessment Questionnaire (CHAQ), and Juvenile Arthritis Quality of life Questionnaire (JAQQ). Examining physicians recorded physician global assessment of disease activity (PGA;VAS 0–100) and active joint count. We used univariate and multivariate regression (controlled for gender) to examine relationships between disease and patient factors and PAQ score at study entry. We used PAQ standard population normative values to compare to subjects (female 2.69, SD 0.62; male 3.0 SD 0.72).Results:We included the first 189 patients enrolled (126 F, 63 M, med age 13 y) from March 2012–November 2013. The early cohort included 81 patients (enrolled median 2.4 mo after diagnosis) and the late cohort included 108 patients (enrolled median 4.8 yr after diagnosis). Active arthritis was found in 88 patients, with a mean of 5.5 active joints (range 1–56). PGA was a mean of 13.45 (range 0–74). Overall mean PAQ score for the JIA patients was 2.55 (SD 0.73, range 1–4). Table describes PAQ scores by sex, disease cohort, and presence of active arthritis. When controlled for sex, PAQ scores were significantly different between new onset and late disease, and between patients with active arthritis compared with no active arthritis. Univariate analyses revealed that higher PAQ score were associated with lower pain (p = 0.02), lower CHAQ score (p &lt; 0.0001), higher JAQQ score (p = 0.0001), lower active joints (p = 0.002) and low PGA (p = 0.003). Multivariate analysis confirmed these associations, with R2 = 0.25, p = 0.000 (girls), R2 = 0.17, p = 0.01 (boys). PAQ scores in patients with JIA Total Female (mean, SD) Female z‐score (mean, SD) Male (mean, SD) Male z‐score (mean, SD) Total 2.5 (0.73) 2.43 (0.66) −0.40 (1.05) 2.77 (0.81) −0.31(1.13) Early disease 2.42 (0.77) 2.29 (0.66) −0.63 (1.05) 2.59 (0.9) −0.44 (1.3) Late disease 2.65 (0.68) 2.53 (0.64) −0.4 (1.04) 2.94 (0.68) −0.07 (0.94) Active joints present 2.36 (0.76) 2.13 (0.59) −0.88 (0.94) 2.77 (0.85) −0.2 (0.85) No active joints 2.71 (0.67) 2.66 (0.6) −0.04 (0.95) 2.77 (0.78) −0.31 (0.78)Conclusion:Children with JIA report significantly lower levels of PA than healthy peers; this was evident in girls, patients with active arthritis, and in early disease. Disease activity, pain, self reported quality of life and functional status may play a role in moderating PA in JIA.

Assessment of Disease Activity in Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is a chronic inflammation of joints in pediatric patients. Assessment of JIA disease activity is very difficult, because children cannot definitely describe their pain by themselves due to development of cognitive function during the pediatric period. Assessment of JIA disease activity is useful for

The JADAS: A Simple and Reliable Tool for the Measurement of Disease Activity in Juvenile Idiopathic Arthritis

The JADAS: A Simple and Reliable Tool for the Measurement of Disease Activity in Juvenile Idiopathic Arthritis

Defining criteria for high disease activity in juvenile idiopathic arthritis based on the Juvenile Arthritis Disease Activity Score

ObjectiveTo determine cutoff values for defining the state of high disease activity (HDA) in juvenile idiopathic arthritis (JIA) using the Juvenile Arthritis Disease Activity Score (JADAS).MethodsFor the selection of cutoff...

PReS13-SPK-1332: Assessment of disease activity and damage in juvenile idiopathic arthritis

A vast array of instruments are available for measuring disease activity in juvenile idiopathic arthritis (JIA). However, due to the high variability in the clinical presentation and course of JIA, no single measure can reliably capture disease activity in all patients. On the other hand, assessment of all measures individually may cause methodological and statistical problems, especially when these measures are employed as endpoints in clinical trials. Several approaches can be followed to achieve a more rational and standardized evaluation. One of these approaches is based on the so-called composite disease activity scores, which are made of a pool of individual measures and are aimed to quantify the absolute level of disease activity by providing one summary number on a continuous scale. Recently, the first composite disease activity score for JIA, named Juvenile Arthritis Disease Activity Score (JADAS), has been developed. In validation analyses, it was found to have good metrologic properties, including the ability to predict the disease outcome. The cutoff values of the JADAS that corresponded with the states of inactive disease and minimal disease activity, or reflected the physician’s, parent’s or child’s subjective rating of remission or the parent’s or child’s satisfaction with the outcome of the illness were established recently. These cutoffs represent an additional clinical tool that, if applied regularly in daily practice, may allow tighter control of therapy, support the optimization of treatment on an individual patient basis, and help prevent the development of joint damage and physical disability. JIA is characterized by prolonged synovial inflammation that may cause irreversible alterations in joint structures. Permanent changes may also occur in extra-articular organs/systems, such as the eye (as a complication of chronic anterior uveitis), or may result from side effects of medications. The Juvenile Arthritis Damage Index (JADI) was devised to enable a thorough detection of articular and extra-articular damage in children with JIA. The JADI is aimed to capture damage, defined as persistent changes in anatomy, physiology, pathology or function, which may be the consequence of previous active disease, side effects of therapy, or co-morbid conditions, is not due to currently active arthritis, and is present for at least 6 months. Damage is often irreversible and cumulative and, thus, damage scores are most frequently expected to increase or remain stable over time. However, because some forms of damage may improve or even resolve in growing children, in some cases scores may decrease. The index is composed of two parts, one devoted to the assessment of articular damage (JADI-A) and one devoted to the assessment of extraarticular damage (JADI-E). In validation analyses, the JADI was found to be feasible and to possess both face and content validity; furthermore, it exhibited good convergent construct validity, excellent reliability (interrater agreement and internal consistency), and strong discriminative validity in a large cohort of JIA patients with long-standing disease.

PReS-FINAL-2063: Proposed criteria for activity, damage and impact of juvenile idiopathic arthritis associated uveitis: consensus effort from the multinational interdisciplinary working group for uveitis in childhood (MIWGUC)

Uveitis is the most common extraartricular manifesation of juvenile idiopathic arthritis (JIA) with an.incidence of approx. 10 - 15 per 100,000 children. Uveitis, a potentially blinding disorder, is present in approx 10-18% of JIA patients, with a wide spectrum of presentations. Despite advances in treatment of childhood arthritis there are no definitions for assessment of activity, damage and impact of JIA associated uveitis.

PReS-FINAL-2132: Defining criteria for high disease activity in juvenile idiopathic arthritis: definition and validation of juvenile arthritis disease activity score cutoffs

Assessment of disease activity is a fundamental component of the clinical evaluation of children with juvenile idiopathic arthritis (JIA) because persistently active disease plays a major role in causing joint damage and physical functional disability. Furthermore, measurement of the level of disease activity over time is important in monitoring the disease course and in assessing the effectiveness of therapeutic interventions.

Assessment of disease activity by patients with juvenile idiopathic arthritis and the parents compared to the assessment by pediatric rheumatologists

BackgroundSelf assessment of arthritis is important for recognition of disease activity and early initiation of therapy. Proper interpretation of physical symptoms is necessary for this. The purpose was to investigate the assessment by patients and parents of disease activity in juvenile idiopathic arthritis (JIA) and to compare their assessments to rheumatologists’ assessments.MethodsPatients and parents assessed 69 joints on a paper homunculus and marked each joint with a different color according to presumed presence of disease: active disease (AD), doubt, and non-active disease (NAD). Their assessments were compared to the rheumatologists’ assessments. If patients and/or parents marked an inflamed joint, it counted as AD. Pain, functional impairment, and disease duration were analyzed to differentiate more precise between true and false positive and true and false negative assessments.ResultsWe collected assessments of 113 patients and/or parents. AD was assessed 54 times, 33 of which were true positives. NAD was assessed 23 times, 22 of which were true negatives. Doubt was expressed 36 times, 9 of which were assessed by the rheumatologist as AD. Sensitivity and specificity of AD was 0.77 and 0.31. Pain and functional impairment scored highest in AD, intermediate in doubt, and lowest in NAD.ConclusionPatients and/or parents seldom missed arthritis but frequently overestimated disease activity. Pain, functional impairment, disease duration, gender, and age did not differentiate between true and false positives for. Patients perceived JIA as active if they experienced pain and functional impairment. To reduce overestimation of the presence of AD we need to improve their understanding of disease activity by teaching them to distinguish between primary symptoms of JIA and symptoms like pain and functional impairment.

Methotrexate polyglutamates in erythrocytes are associated with lower disease activity in juvenile idiopathic arthritis patients

ObjectiveTo determine association of erythrocyte methotrexate polyglutamates (MTX-PG) with disease activity and adverse effects in a prospective juvenile idiopathic arthritis (JIA) cohort.MethodsOne hundred and thirteen JIA patients were followed from...

Sleep and its relationship to pain, dysfunction, and disease activity in juvenile idiopathic arthritis

The objective of this study was to determine the sleep abnormalities that may exist in Moroccan children with juvenile idiopathic arthritis (JIA) and their relationship to pain, dysfunction, and disease activity. Case control study including 47 patients diagnosed with JIA, according to the criteria of the International League of Associations for Rheumatology (ILAR), and 47 healthy children, age and sex matched. Sleep was assessed by Children's Sleep Habits Questionnaire (CSHQ). All parents have filled the 45 items of the CSHQ and grouped into eight subscales: bedtime resistance, sleep onset delay, sleep duration, sleep anxiety, sleep-disordered breathing, night awakenings, parasomnias, and morning awakening/daytime sleepiness. The disease activity was assessed by the number of painful joints, swelling joints, erythrocyte sedimentation rate, c-protein reactive, and Juvenile Arthritis Disease Activity Score (JADAS). Functional assessment was based on the value of Childhood Health Assessment Questionnaire. Pain was assessed by visual analog scale pain. Forty-seven patients were included, with 28 males (59.6%). Children with JIA had a total score of CSHQ significantly higher than the control cases (p < 0.0001); significant differences were also found in the subscale sleep onset delay, sleep anxiety, sleep-disordered breathing, night awakenings, and parasomnias with a p value of <0.0001, 0.034, <0.0001, 0.001, and 0.00, respectively. Significant association was found between the CSHQ total score and visual analog scale (VAS) physician activity (p = 0.016) and JADAS (p = 0.05). There was a correlation between the sleep-disordered breathing and JADAS (p = 0.04). Sleep onset delay was associated with VAS patient pain (p = 0.05), as nocturnal awakenings and VAS patient pain (p = 0.016). Finally, parasomnias and physician's VAS activity (p = 0.015) and VAS patient pain (p = 0.03) were also correlated. This study suggests that sleep abnormalities are common in children with JIA. Strategies to improve sleep should be studied as a possible tool of improving the quality of life of children with rheumatic disease.

Temporomandibular signs, symptoms, joint alterations and disease activity in juvenile idiopathic arthritis – an observational study

BackgroundJuvenile idiopathic arthritis (JIA) is a heterogeneous disease that frequently affects also the temporomandibular joint (TMJ) and associated structures. The main aim of this observational study was to describe systematically orofacial clinical signs and subjective symptoms in JIA patients, classified according to the International League of Associations for Rheumatology (ILAR) criteria, and to relate the findings to disease activity and radiological TMJ condyle lesions.MethodsThe study was a retrospective evaluation of dental and medical records in consecutive JIA patients referred to one of three dental specialist clinics in Sweden during an eight-year period. Data concerning temporomandibular signs, symptoms and general disease activity were collected and condylar alterations were judged on panoramic radiographs.ResultsAll ILAR categories of JIA were represented among the 266 referrals included in the study. The distribution of patients among categories resembled the pattern seen in epidemiological studies. Persistent oligoarthritis was the largest category with 36.5% of the patients. Temporomandibular clinical signs (mild, moderate or severe) occurred in 57.7% to 92.0%, and subjective symptoms (mild or severe) in 32.0% to 76.0% of the patients in all categories. Patients in the juvenile psoriatic arthritis category had the largest number of orofacial signs and symptoms, and patients in the persistent oligoarthritis category had the fewest signs and symptoms. There were significant associations between clinical signs as well as subjective symptoms and overall disease activity. Half of all the patients had undergone panoramic examinations and 37.9% of those were judged to have condylar alterations after a mean of 2.9 years after onset. No associations between radiological findings and variables, such as signs, symptoms or disease activity, were found.ConclusionsTemporomandibular signs and symptoms can be expected to a varying degree, including severe cases, in all JIA categories. Clinical and subjective orofacial involvement appears to be related to disease activity but not to condylar lesions.

Contrast-enhanced MRI compared with the physical examination in the evaluation of disease activity in juvenile idiopathic arthritis

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. 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. 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. 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. • 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.