Aims: To determine the test-retest reliability and responsiveness of a new computerized 20-item pain measure, SUPER-KIDZ, in children with juvenile idiopathic arthritis (JIA). Methods: A single centre prospective cohort study of JIA patients aged 8-18 years was performed. For each SUPER-KIDZ item, test-retest reliability analysis was done in patients expected to have stable pain, and responsiveness was evaluated after intra-articular steroid injection(s). Results: Fifty-one subjects were included. Good internal consistency (α=0.73-0.92) was demonstrated for the 3 SUPER-KIDZ domains. Acceptable test-retest reliability (intraclass correlation coefficient or kappa ≥0.80) was found for 15 SUPER-KIDZ items. At 2 weeks post-injection, 16 items were responsive to change in pain (standardized response mean=0.66-0.82, significant Wilcoxon signed rank and linear mixed model). Conclusions: The majority of the SUPER-KIDZ items have acceptable test-retest reliability and responsiveness properties. If validity is demonstrated, this measure could be implemented as a standardized comprehensive pain tool for JIA patients.

Aims: To determine the test-retest reliability and responsiveness of a new computerized 20-item pain measure, SUPER-KIDZ, in children with juvenile idiopathic arthritis (JIA). Methods: A single centre prospective cohort study of JIA patients aged 8-18 years was performed. For each SUPER-KIDZ item, test-retest reliability analysis was done in patients expected to have stable pain, and responsiveness was evaluated after intra-articular steroid injection(s). Results: Fifty-one subjects were included. Good internal consistency (α=0.73-0.92) was demonstrated for the 3 SUPER-KIDZ domains. Acceptable test-retest reliability (intraclass correlation coefficient or kappa ≥0.80) was found for 15 SUPER-KIDZ items. At 2 weeks post-injection, 16 items were responsive to change in pain (standardized response mean=0.66-0.82, significant Wilcoxon signed rank and linear mixed model). Conclusions: The majority of the SUPER-KIDZ items have acceptable test-retest reliability and responsiveness properties. If validity is demonstrated, this measure could be implemented as a standardized comprehensive pain tool for JIA patients.

Mia Alessandra Nieto is an 8-year old living with Juvenile Idiopathic Arthritis (JIA) in Austin, Texas. When she was diagnosed at 10 months, she was the youngest child ever diagnosed with JIA in the state of Texas. However, it took 37 days to confirm her condition because there is an immense lack on knowledge in the field of pediatric rheumatology among general practitioners despite the fact that JIA is the most prevalent chronic condition in children in the United States with over 300,000 diagnosed. This is an overview of Mia’s story, along with information regarding the lack of knowledge on the condition not only in the general population but mainly and more importantly among the medical professionals in the United States. / text

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Charles University, Faculty of Pharmacy in Hradec Králové Department of Pharmacology & Toxicology Student: Daniel Diviš Supervisors: Prof. Dr. Florence Apparailly, Directrice de Recherche Prof. PharmDr. Petr Pávek, Ph.D. (formal tutor) Title of diploma thesis: The role of miR-150 in the physiopathology of oligoarticular juvenile idiopathic arthritis Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatoid disease affecting children, and its pathological mechanisms are still poorly understood. Innate and adaptive immunity including myeloid cells play a major role in these processes. Epigenetic deregulations along with non-coding microRNAs have been reported in many inflammatory diseases. Moreover, preliminary results obtained by the research group of Prof. Florence Apparailly showed accumulation of intermediate monocytes along with the high expression of miR-150 in the synovial fluid of children affected by oligoarticular JIA. Based on these findings a hypothesis has been postulated suggesting that miR-150 could have a role in the pathogenesis of this disease and in the regulation of monocyte differentiation and function. To study the impact of miR-150 on monocytes from the peripheral blood of healthy donors, transfection experiments were performed to neutralize miR-150. The...

Introdução: A Artrite Idiopática Juvenil (AIJ) inicia-se principalmente no intervalo etário de oito a catorze anos, podendo estar associada a déficit de crescimento e atraso puberal. O estadiamento de Tanner e a avaliação ultrassonográfica da pelve podem detectar atrasos no desenvolvimento puberal. Objetivos: Comparar a maturação sexual das meninas com AIJ a controles saudáveis, por meio da avaliação clínica dos estágios de Tanner e dos parâmetros da ultrassonografia (US) pélvica e relacionar esses achados aos níveis de hormônios sexuais e fatores relacionados à doença. Comparar, também, os dados antropométricos e a idade da menarca entre os dois grupos e relacioná-los a fatores de risco ligados à AIJ. Delineamento: Estudo transversal com grupo controle Metodologia: O estudo foi realizado com 44 meninas com AIJ e 59 controles com idades entre seis e dezoito anos incompletos, sem uso de glicocorticoides há no mínimo seis meses e sem outras doenças crônicas concomitantes. O diagnóstico de AIJ foi realizado de acordo com critérios da Liga Internacional das Associações de Reumatologia (ILAR). Foi realizada avaliação antropométrica, e a maturação sexual foi avaliada por meio dos estádios de Tanner. A US pélvica abdominal foi utilizada para avaliar as medidas do útero dos ovários e o índice de pulsatilidade das artérias uterinas (IP). Foram medidos níveis hormonais de FSH, LH, estrógeno, progesterona e IGF-1 nas meninas com AIJ. Resultados: Os parâmetros da US pélvica foram correlacionados aos estágios de Tanner no grupo controle (p <0,001). Todas as medidas de útero e ovários foram menores nas meninas com AIJ quando comparadas ao grupo controle. A média do IP das artérias uterinas foi maior nas meninas com AIJ. Estratificando-se por idade, o volume do útero foi menor nas meninas com AIJ na faixa etária de 10-11 anos (p < 0,004) e 14-15 anos (p=0,042), e a relação corpo/cérvice AP foi menor no intervalo de 10-11 anos (p=0,007). Os níveis de LH e estradiol foram fortemente relacionados ao aumento dos órgãos pélvicos (p<0,001) e inversamente com IP médio das artérias uterinas (p<0.01). O escore z do IMC e da estatura foi menor nas meninas com AIJ em relação ao grupo controle (p=0,032 e p=0,041 respectivamente). As meninas com AIJ poliarticular e com maior dose cumulativa de glicocorticoide apresentaram a maior chance de ter baixa estatura. Não houve diferença entre os grupos AIJ e controles com relação à idade da menarca. A altura final e a diferença entre essa altura e a altura-alvo familiar não foi diferente entre as meninas com AIJ e as do grupo controle. Conclusão: Nosso estudo mostrou que, mesmo sem uso de glicocorticoide há mais de seis meses, as crianças com formas mais graves de AIJ e que necessitaram de doses maiores de glicocorticoide estão mais suscetíveis ao retardo no crescimento e atraso no início da puberdade. A US pélvica demonstrou ser um exame sensível para avaliação da maturação sexual de meninas, identificando atrasos nas meninas com AIJ não percebidas por meio da avaliação clínica pelos estágios de Tanner. / Introduction: Juvenile Idiopathic Arthritis (JIA) manifests at the 8 to 14-year age span and is often associated with growth deficit and puberty delay. Tanner staging and pelvic ultrasonography (US) can be used to detect pubertal delays. Objectives: To compare sexual maturation in JIA girls and healthy controls (HC) by clinical evaluation with Tanner stages and pelvic US parameters and to correlate these findings with sexual hormone levels and disease related factors. Additionally, to compare anthropometric data and menarche age between groups and to correlate such findings with disease related risk factors in JIA patients. Study design: Cross-sectional study. Methods: 44 JIA and 59 healthy girls, aged six to 18 years, free of steroid use in the last six months and with no concomitant chronic diseases were included the study. JIA was diagnosed after the International League of the Rheumatology Associations (ILAR) criteria. Anthropometric and sexual development evaluations by Tanner staging were performed Pelvic US was performed to measure uterus, endometrial thickness, ovaries and uterine artery pulsatility index. Sexual hormone levels were measured in JIA girls. Results: Pelvic US parameters correlated with Tanner stages in HC (p <0.001). All uterine and ovarian measurements were smaller in JIA girls than in HC. Mean uterine artery PI was greater in JIA girls. Uterine volume was smaller in JIA girls at the 10 to 11 (P=0.004) and 14 to 15 year (p=0.042) age strata and the anteroposterior body cervix ratio was smaller in JIA girls the 10 to 11 year stratum (p=0.007). LH and estradiol levels were strongly correlated with pelvic organ size. (P<0.001) and inversely correlated with mean uterine artery PI (p<0.01) BMI and height for age z scores were smaller in JIA girls than in HC. Polyarticular JIA girls and those that had received greater steroid doses had the largest chance to have short stature. There was no difference between JIA girls and HC regarding menarche age. Final height and the final height/target height difference was not different between JIA girls and HC. Conclusion: The current study showed that JIA girls from the most severe subtypes and those that had received the largest steroid cumulative doses were more susceptible to growth and puberty delays, even six months after drug withdrawal. Pelvic US was a sensitive tool in detecting sexual development in girls, being able to identify puberty delays, unsuspected by Tanner stage evaluation.

Aim: To describe the longitudinal active joint count (AJC) trajectories in juvenile idiopathic arthritis (JIA). Methods: A retrospective cohort study at two Canadian centres was performed. The longitudinal trajectories of AJC were described using latent growth curve modeling. The association of baseline characteristics stratified by trajectory group was examined by univariate methods. Results: Data were analyzed for 659 children diagnosed with JIA between 1990/03-2009/09. A maximum of 10 years of follow-up data were included in the analysis. Participants were classified into 5 statistically and clinically distinct AJC trajectories by latent GCM. Conclusions: Using a novel longitudinal statistical method we were able to classify patients with JIA based on their pattern of AJC over time. The trajectory classes need to be examined for their relationship to important genetic and biological predictors. Identification of patterns of disease course is important in working towards the development of a clinically relevant outcome-based classification system in JIA.

Aim: To describe the longitudinal active joint count (AJC) trajectories in juvenile idiopathic arthritis (JIA). Methods: A retrospective cohort study at two Canadian centres was performed. The longitudinal trajectories of AJC were described using latent growth curve modeling. The association of baseline characteristics stratified by trajectory group was examined by univariate methods. Results: Data were analyzed for 659 children diagnosed with JIA between 1990/03-2009/09. A maximum of 10 years of follow-up data were included in the analysis. Participants were classified into 5 statistically and clinically distinct AJC trajectories by latent GCM. Conclusions: Using a novel longitudinal statistical method we were able to classify patients with JIA based on their pattern of AJC over time. The trajectory classes need to be examined for their relationship to important genetic and biological predictors. Identification of patterns of disease course is important in working towards the development of a clinically relevant outcome-based classification system in JIA.

BACKGROUND: Parents of children with juvenile idiopathic arthritis (JIA) are often forced to make trade-offs between the effectiveness, convenience, safety, and cost of drug treatments for their child. METHODS: A discrete choice experiment was administered to parents of children with JIA to determine their preferences for drug treatments. Multinomial logit regression was used to estimate part-worth utilities and willingness-to-pay. RESULTS: Participation in daily activities was the most important attribute, followed by child reported pain. Child age, gender, years with JIA, and household income had the greatest impact on preferences. Parents’ were willing to pay $2,080 to switch from a drug representing methotrexate to etanercept (95% CI $698, $4,065). CONCLUSIONS: Parents of children with JIA have the highest maximum willingness-to-pay for drug treatments that improve daily functioning and reduce pain. Cost is a significant factor in the decisions that parents make surrounding the best treatment for a child.

BACKGROUND: Parents of children with juvenile idiopathic arthritis (JIA) are often forced to make trade-offs between the effectiveness, convenience, safety, and cost of drug treatments for their child. METHODS: A discrete choice experiment was administered to parents of children with JIA to determine their preferences for drug treatments. Multinomial logit regression was used to estimate part-worth utilities and willingness-to-pay. RESULTS: Participation in daily activities was the most important attribute, followed by child reported pain. Child age, gender, years with JIA, and household income had the greatest impact on preferences. Parents’ were willing to pay $2,080 to switch from a drug representing methotrexate to etanercept (95% CI $698, $4,065). CONCLUSIONS: Parents of children with JIA have the highest maximum willingness-to-pay for drug treatments that improve daily functioning and reduce pain. Cost is a significant factor in the decisions that parents make surrounding the best treatment for a child.