Juvenile Idiopathic Arthritis: Diagnosis and Treatment

Juvenile idiopathic arthritis is a broad term that describes a clinically heterogeneous group of arthritides of unknown cause, which begin before 16 years of age. This term encompasses several disease categories, each of which has distinct presentation, clinical manifestations, and, presumably, genetic background and etiopathogenesis. Although none of the available drugs has curative potential, prognosis has greatly improved as a result of substantial progresses in disease management. The most important new development has been the introduction of the biologic medications, which constitute a valuable treatment option for patients who are resistant to conventional antirheumatic agents. Further insights into the disease pathogenesis and treatment will be provided by the continuous advances in understanding of the mechanisms related to the immune response and inflammatory process, and by the development of new drugs that are capable of selectively inhibiting single molecules or pathways

Clinical outcome measures in juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality of life. In order to optimize JIA management, the paediatric rheumatologist has begun to regularly use measurements of disease activity developed, validated and endorsed by international paediatric rheumatology professional societies in an effort to monitor the disease course over time and assess the efficacy of therapeutic interventions in JIA patients.A literature review was performed to describe the main outcome measures currently used in JIA patients to determine disease activity status.The Juvenile Disease Activity Score (JADAS), in its different versions (classic JADAS, JADAS-CRP and cJADAS) and the validated definitions of disease activity and response to treatment represent an important tool for the assessment of clinically relevant changes in disease activity, leading more and more to a treat-to-target strategy, based on a tight and thorough control of the patient condition. Moreover, in recent years, increasing attention on the incorporation of patient-reported or parent-reported outcomes (PRCOs), when measuring the health state of patients with paediatric rheumatic diseases has emerged.We think that the care of JIA patients cannot be possible without taking into account clinical outcome measures and, in this regard, further work is required

Predictors of polyarticular extension in oligoaricular-onset juvenile idiopathic arthritis

Predictors of polyarticular extension in oligoaricular-onset juvenile idiopathic arthriti

The conundrum of juvenile psoriatic arthritis

Juvenile psoriatic arthritis (JPsA) has provided paediatric rheumatologists with a controversial topic for many years. The principal area of contention centres on the discordance between its treatment as a single diagnostic category in current classification schemes and the demonstration of its heterogeneous nature. A further point of debate is the distinctiveness of JPsA as an entity. Owing to these uncertainties, the concept of JPsA has evolved over the years and there have been several changes in its definition and diagnostic criteria. Recently, strong evidence has been provided that the spectrum of JPsA include at least two distinct subgroups, one that has the same characteristics as early-onset ANA-positive JIA, and another that is part of the spectrum of spondyloarthropathies and resembles the forms of psoriatic arthritis in adults that belong to the same disease family. These findings call for a revision of the classification of childhood arthritis, that refutes the assumptions that children with JPsA constitute a single homogeneous population and that JPsA should be considered an individual disease entity

A new short and simple health-related quality of life measurement for paediatric rheumatic diseases: initial validation in juvenile idiopathic arthritis

Objective. To develop and validate a new short and simple measure of health-related quality of life (HRQL) in children with juvenile idiopathic arthritis (JIA).Methods. The Paediatric Rheumatology Quality of Life Scale (PRQL) is a 10-item questionnaire that explores HRQL in two domains: physical health (PhH) and psychosocial health (PsH). Validation of the parent proxy report and child self-report versions of the instrument was accomplished by evaluating 472 JIA patients and similar to 800 healthy children. Validation analyses included assessment of feasibility, face and content validity; construct and discriminative ability; internal structure and consistency; test-retest reliability; responsiveness to clinical change; and minimal clinically important difference.Results. The PRQL was found to be feasible and to possess both face and content validity. The PRQL score correlated in the predicted range with most of the other JIA outcome measures, thereby demonstrating good construct validity, and discriminated well between different levels of disease severity. Assessment of internal structure (factor analysis) revealed that the PhH and PsH subscales identify two unambiguously separated domains. The internal consistency (Cronbach's alpha) was 0.86. The intraclass correlation coefficient for test-retest reliability was 0.91. The PRQL revealed fair responsiveness, with a standardized response mean of 0.67 in improved patients. Overall, the PRQL appeared to be more able to capture physical HRQL than psychosocial HRQL.Conclusion. The PRQL was found to possess good measurement properties and is, therefore, a valid instrument for the assessment of HRQL in children with JIA. This tool is primarily proposed for use in standard clinical care

A prediction rule for polyarticular extension in oligoarticular-onset juvenile idiopathic arthritis

To search for predictors of polyarticular extension in children with oligoarticular-onset juvenile idiopathic arthritis (JIA) and to develop a prediction model for an extended course

Oral or subcutaneus methotrexate: comparison of the efficacy in inducing sustained disease remission in children with oligoarticular JIA

Introduction: Methotrexate (MTX) is widely adopted as a first line treatment in moderate to severe forms of juvenile idiopathic arthritis (JIA), when NSAIDs and intra-articular corticosteroid injections are not sufficient to control joint disease. MTX is generally prescribed at 10-15 mg/m2 weekly and its administration can be either oral or parenteral (subcutaneous (SC) or intramuscular). Contrasting evidence is available in the literature about the difference in efficacy and safety of MTX, according to the route of administration. Objectives: Aim of the study is to compare the efficacy of oral versus SC MTX in inducing sustained disease remission in children with oligoarticular JIA enrolled in two prospective cohorts. Methods: Children with oligoarthritis included in 3 prospective studies were considered for inclusion: a) the TRIMECA trial (1), b) the MD-Paedigree study (2), c) the PharmaChild registry. Patient evaluated at the IRCCS Istituto Giannina Gaslini and at the Ospedale Pediatrico Bambino Gesù were included if they had received methotrexate treatment as a first line systemic medication within 6 months after disease onset and if a follow up of at least 12 month after treatment initiation was available. Patients were then grouped according to the route of MTX administration. Baseline demographic and disease features were compared between the 2 groups. Efficacy was assessed by comparing the rate of inactive disease (ID) and clinical remission on medication (CRM) at 12 months, the rate of patients changing the route of MTX administration or requiring a biologic medication due to treatment failure. Safety was assessed by comparing the frequency of treatment interruption due to side effects of MTX. Results: 79 patients were included in the study: 43 received oral MTX, 36 received SC MTX. At treatment initiation, disease duration was not different in the two groups; children receiving SC MTX were older at baseline (4.6 yrs vs. 2.5 yrs) and at disease onset (4.2 yrs vs. 2.3 yrs). Disease activity was comparable in the 2 groups, with a median of 2 active joints in both groups. Median MTX dose was 14.4 mg/m2 for oral MTX group and 15.2 mg/m2 for SC MTX (Mann-Whitney U test, p < 0.01). At 12 months, children receiving SC MTX achieved more frequently ID (84.9% vs 43.8%, Chi squared test p < 0.001) and CRM (54.5% vs 28.3%, p = 0.002). Patients in SC MTX group were more often prescribed a biologic medication (22.9% vs 6.5%, p = 0.024), but none of them were switched to the oral administration while 37% of children in oral MTX group were turned to SC MTX. One patient in both groups had MTX treatment suspended due to side effects. Conclusion: Our preliminary results support the evidence of an increased efficacy of MTX in inducing sustained disease remission when it is administered subcutaneously

Analysis of arthritis flares after achievement of inactive disease with methotrexate monotherapy in juvenile idiopathic arthritis

To investigate the frequency of arthritis flare and factors affecting occurrence of flare in children with juvenile idiopathic arthritis (JIA) who achieved inactive disease (ID) with methotrexate (MTX) monotherapy