The Brazilian Portuguese 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 Brazilian Portuguese language. The reading comprehension of the questionnaire was tested in 10 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 231 JIA patients (14.7% systemic, 43.3% oligoarticular, 22.5% RF negative polyarthritis, 19.5% other categories) and 72 healthy children, were enrolled in three centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Brazilian Portuguese 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.

Progression of articular and extraarticular damage in oligoarticular juvenile idiopathic arthritis.

OBJECTIVES: Evaluate damage in oligoarticular JIA, estimating its frequency, risks and probability over time. METHODS: A cross-sectional and retrospective analysis of Juvenile Arthritis Damage Index (JADI) scoring, with both articular and extraarticular components, active joint count, disability index by CHAQ and Steinbrocker class, physician's global assessment, child's pain and overall well-being visual analogue scale (VAS), was conducted in patients with oligoarticular JIA. Damage risk factors were estimated by univariate analysis and by generalised linear model. The probability of damage over time was estimated by survival analysis and damage progression rates were calculated by hazard function. RESULTS: Seventy-five JIA cases were assessed, 89.3% persistent and 10.7% extended oligoarthritis, with median follow-up duration 1.7 years (IQR 1.3-3.1). Damage occurred in 38.7%. JADI-A correlated moderately only with the number of limited joints (rs= 0.50, p<0.0001). Female sex (OR 3.5, 95% CI 1.0-11.6), DMARD use (OR 3.9, 95%CI 1.0-15.0) and knee involvement (OR 4.2, 95%CI 1.3-13.5) were significantly associated with joint damage, whereas only joint steroid injection was associated with extraarticular damage (OR 5.9, 95% CI 1.8-19.3). Damage probability at 5 years was 50% for JADI-A, and 57% for JADI-E. Calculated hazard rates each year were 16.1% and 16.3%, for JADI-A and JADI-E, respectively. CONCLUSIONS: Sex, DMARD use and knee involvement were associated with joint damage, whereas only joint steroid injection was associated with extraarticular damage, which progressed at stable rates over ten years.

Safety and immunogenicity of the quadrivalent human papillomavirus vaccine in patients with juvenile dermatomyositis: a real-world multicentre study.

BACKGROUND: Concerns about the safety and efficacy of vaccines in patients with autoimmune diseases (AID) have led to contradictions and low vaccination coverage in this population, who are at a higher risk of infections, including by human papillomavirus (HPV). Although HPV vaccines have been recommended for immunocompromised patients, there is still a lack of data to support its use for AID patients, such as juvenile dermatomyositis (JDM) patients. The aim of this study was to assess the safety and immunogenicity of the quadrivalent HPV (qHPV) vaccine in a cohort of JDM patients. METHODS: JDM patients aged from 9 to 20 years and healthy controls (HC) were enrolled to receive a 3-dose schedule of qHPV vaccine from March/2014 to March/2016. Study visits were performed before the first dose, 1 month after the second and third doses, and 6 months after the third dose. Participants completed a diary of possible adverse events for 14 days following each dose of vaccination (AEFV). Disease activity and current therapy were analyzed at each visit for JDM patients. In addition, serum samples from all participants were collected to test antibody concentrations against HPV16 and 18 at each visit. Participant recruitment was conducted in ten Brazilian centres. From 47 eligible JDM patients and 41 HC, 42 and 35, respectively, completed the 3-dose schedule of the vaccine, given that five JDM patients and two HC had received doses prior to their inclusion in the study. RESULTS: The AEFVs presented by the participants were mild and in general did not differ between JDM and HC groups. No severe AEFVs were related to the vaccination. Disease activity was stable, or even improved during the follow-up. One month after the third dose of the vaccine the JDM group presented seropositivity of 100% for HPV16 and 97% for HPV18, similarly to the HC group, who presented 100% for both serotypes (p = 1.000). Six months after the third dose the seropositivity for the patient group was 94% for both HPV types. CONCLUSIONS: The HPV vaccination in this cohort of JDM patients was safe and immunogenic. Since the seropositivity against HPV16 and 18 was very high after the 3-dose schedule, this regimen should be recommended for JDM patients. TRIAL REGISTRATION: Brazilian Clinical Trials Registry, number: RBR-9ypbtf . Registered 20 March 2018 - Retrospectively registered.

Health-related quality of life of patients with juvenile dermatomyositis: results from the Pediatric Rheumatology International Trials Organisation multinational quality of life cohort study.

OBJECTIVE: To investigate the health-related quality of life (HRQOL) change over time, as measured by the Child Health Questionnaire (CHQ), and its determinants in patients with active juvenile dermatomyositis (DM). METHODS: We assessed patients with juvenile DM at both baseline and 6 months of followup, and healthy children age < or =18 years. Potential determinants of poor HRQOL included demographic data, physician's and parent's global assessments, muscle strength, functional ability as measured by the Childhood Health Assessment Questionnaire (C-HAQ), global disease activity assessments, and laboratory markers. RESULTS: A total of 272 children with juvenile DM and 2,288 healthy children were enrolled from 37 countries. The mean +/- SD CHQ physical and psychosocial summary scores were significantly lower in children with juvenile DM (33.7 +/- 11.7 versus 54.6 +/- 4.1) than in healthy children (45.1 +/- 9.0 versus 52 +/- 7.2), with physical well-being domains being the most impaired. HRQOL improved over time in responders to treatment and remained unchanged or worsened in nonresponders. Both physical and psychosocial summary scores decreased with increasing levels of disease activity, muscle strength, and parent's evaluation of the child's overall well-being. A C-HAQ score >1.6 (odds ratio [OR] 5.06, 95% confidence interval [95% CI] 2.03-12.59), child's overall well-being score >6.2 (OR 5.24, 95% CI 2.27-12.10), and to a lesser extent muscle strength and alanine aminotransferase level were the strongest determinants of poor physical well-being at baseline. Baseline disability and longer disease duration were the major determinants for poor physical well-being at followup. CONCLUSION: We found that patients with juvenile DM have a significant impairment in their HRQOL compared with healthy peers, particularly in the physical domain. Physical well-being was mostly affected by the level of functional impairment.