S Dalprà

A New Approach to Clinical Care of Juvenile Idiopathic Arthritis: The Juvenile Arthritis Multidimensional Assessment Report

Objective. To develop and test a new multidimensional questionnaire for assessment of children with juvenile idiopathic arthritis (JIA) in standard clinical care. Methods. The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) includes 15 parent or patient-centered measures or items that assess well-being, pain, functional status, health-related quality of life, morning stiffness, disease activity, disease status and course, joint disease, extraarticular symptoms, side effects of medications, therapeutic compliance, and satisfaction with illness outcome. The JAMAR is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 7–18 years for use as a self-report. From March 2007 to September 2009, the questionnaire was completed by the parents of 618 children with JIA in 1814 visits and by 332 children in 749 visits. Results. The JAMAR was found to be feasible and to possess face and content validity. All parents and children reported that the questionnaire was simple and easy to understand. Completion and scoring appeared to be quick, requiring < 15 minutes. There were very few missing data. Parents’ proxy-reported and children’s self-reported data were remarkably concordant. The JAMAR provided thorough information for the study patients about recent medical history and current health status. It performed similarly across different children’s ages and characterized the level of disease activity and disability well. Conclusion. The development of the JAMAR introduces a new approach in pediatric rheumatology practice. This new questionnaire may help enhance the quality of care of children with JIA.

Antinuclear antibody–positive patients should be grouped as a separate category in the classification of juvenile idiopathic arthritis

OBJECTIVE We undertook this study to test the hypothesis that in the International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA), patients with similar characteristics can be classified into different categories. We sought to investigate whether antinuclear antibody (ANA)-positive patients having disease in the ILAR categories of oligoarthritis, rheumatoid factor-negative polyarthritis, psoriatic arthritis, and undifferentiated arthritis share homogeneous features and to compare these features with those of ANA-negative patients having the same categories of disease. METHODS We identified JIA patients who had been followed up during a 22-year period. ANA positivity was defined as ≥2 positive results at a titer of ≥1:160. Demographic and clinical features were recorded retrospectively and compared between ANA-positive and ANA-negative patients. RESULTS Of a total of 971 patients, 711 were ANA positive, 149 were ANA negative, and 111 had an indeterminate ANA status. Patients with indeterminate ANA status were excluded. ANA-positive patients in the different ILAR categories were similar in terms of age at disease presentation, female-to-male ratio, and frequency of asymmetric arthritis and iridocyclitis. Compared with ANA-positive patients, the ANA-negative group was older at disease presentation and had a lower prevalence of females, a lower frequency of iridocyclitis and asymmetric arthritis, a greater number of affected joints over time, and a different pattern of arthritis. The close relationship between the presence of ANAs and younger age at disease presentation, female predominance, asymmetric arthritis, development of iridocyclitis, lower number of affected joints over time, and lack of hip involvement was also confirmed by multivariate and multiple correspondence analysis. CONCLUSION Our findings substantiate the hypothesis that ANA-positive patients classified into different JIA categories by current ILAR criteria constitute a homogeneous patient population.

Development and testing of a hybrid measure of muscle strength in juvenile dermatomyositis for use in routine care

To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing in 8 muscles (MMT‐8) and the Childhood Myositis Assessment Scale (CMAS) but is more comprehensive than the former and more feasible than the latter.

A controlled trial of intra-articular corticosteroids with or without methotrexate in oligoarticular juvenile idiopathic arthritis

In contrast with the numerous controlled trials conducted in polyarticular or systemic juvenile idiopathic arthritis (JIA), little evidence-based information is available for oligoarticular JIA. As a result, the management of children with this subtype, which is the most prevalent in Western countries, is largely empiric. Intra-articular corticosteroid (IAC) injection is the therapy of first choice for oligoarthritis in many pediatric rheumatology centers. However, although IAC injections are usually highly efficacious, relapses of synovitis are common and sometimes occur only a few months after the procedure. It is still unclear whether concomitant administration of methotrexate (MTX) may increase and prolong the effectiveness of IAC injections.

Time of onset of iridocyclitis (IC) in children with juvenile idiopathic arthritis (JIA)

Methods 1050 JIA patients seen between 1985 and 2007 were identified. 172 patients (16.4%) had IC. 6 patients with enthesitis-related arthritis with acute IC, 4 patients who developed IC before arthritis onset and 2 patients in whom date of IC onset was unknown were excluded. Of the remaining 160 patients, 108 (67.5%) had oligoarthritis, 36 (22.5%) RF-negative polyarthritis, 9 (5.6%) undifferentiated arthritis, 6 (3.8%) psoriatic arthritis, 1 (0.6%) systemic arthritis. Of the 158 patients who had ANA tested, 144 (91.1%) were positive (≥ 1:160), 8 (5.1%) low-positive (≤ 1:80) or doubtful, 6 (3.8%) negative. Results The cumulative proportion of patients who developed IC over time is shown in figure 1 and table 1.

PReS-FINAL-2011: Preliminary validation of a new hybrid measure of muscle strength for juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a multisystem vasculopathic disease characterized by muscle inflammation that causes symmetrical muscle weakness. Assessment of muscle strength is, therefore, a fundamental component of the clinical evaluation of children with JDM. This assessment is traditionally made using the 8-muscle Manual Muscle Testing (MMT) and the Childhood Myositis Assessment Scale (CMAS). However, the MMT does not cover all muscles or muscle groups affected in JDM, namely abdominal muscles. Although the CMAS is more comprehensive than the MMT, it is lengthy and, therefore, may not be feasible in a busy clinical setting or when a physical therapist is not available.

Is it worth allowing the presence of morning stiffness in the definition of inactive disease in juvenile idiopathic arthritis

Morning stiffness is a major symptom of juvenile idiopathic arthritis (JIA) and it is usually associated with active disease. However, it is common view that children with disease quiescence may have some degrees of residual morning stiffness. The 2004 preliminary criteria for inactive disease (ID) in JIA did not include the assessment of morning stiffness, whereas the 2011 revision of the criteria has allowed the presence of morning stiffness lasting ≤ 15 minutes. However, it is still unknown whether the disease status of children with ID who have or do not have morning stiffness is comparable.

PReS-FINAL-2176: Declines in levels of disease activity and physical disability in children with juvenile idiopathic arthritis seen in standard clinical care over the last 25 years

Over the last 3 decades there have been important advances in the management of juvenile idiopathic arthritis (JIA), which include the introduction of methotrexate and, later on, the tendency toward its earlier initiation, the widespread use of intra-articular corticosteroid injections, and, more recently, the availability of the biologic response modifiers. Although this therapeutic progress is likely to have led to a marked improvement in the outlook of children with JIA, the prognostic impact of the newer therapeutic modalities is still poorly documented.

Children with juvenile idiopathic arthritis currently followed in a tertiary care setting have a better psychosocial well-being than healthy peers

Methods 669 parents of children with JIA, 398 children with JIA, 801 parents of HC, and 796 HC completed independently the Pediatric Rheumatology Quality of Life scale (PRQL) (Filocamo et al. Rheumatology 2010). Children with JIA and HC who completed the questionnaire were aged > 7-8 years. The PRQL is a 10-item questionnaire that includes 2 subdimensions, physical health (PhH) and psychosocial health (PsH), each composed of 5 items. The total PRQL score ranges from 0 to 30, with higher scores indicating worse HRQOL. A separate score for the PhH and PsH subscales (range 0–15) can be calculated.

Do parent's global rating of well-being and disease activity of children with juvenile idiopathic arthritis yield different information?

Background The parents of children with juvenile idiopathic arthritis (JIA) are traditionally asked to provide a global assessment of disease status by rating the childs overall level of well-being (WB) on a 10-cm visual analogue scale (VAS). However, it has been advised that this measure does not provide a precise assessment of DA because it is largely affected by the presence of disease damage. For this reason, it has been suggested to substitute the VAS for WB with a VAS assessing specifically the level of DA.