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Discordance in perception of disease activity between adolescent patients with lupus and their providers may influence disease outcomes. We found that patients endorsed higher perceptions of disease activity than providers. Discordance was present at all levels of disease activity, particularly in patients with high activity, nephritis, and/or taking corticosteroids or mycophenolate mofetil.

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OBJECTIVE: To develop and validate a diagnostic prediction model that can distinguish between juvenile idiopathic arthritis (JIA) and chronic musculoskeletal pain syndrome (CMPS) based on patient-reported outcomes. STUDY DESIGN: This retrospective cohort study evaluated whether the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) performs well in distinguishing JIA from CMPS. We analyzed JAMARs completed by 287 patients at their first visit to the pediatric rheumatology department of Wilhelmina Children's Hospital in Utrecht, The Netherlands. Relevant JAMAR items for predicting a diagnosis of JIA were selected in a penalized multivariable model suitable for clinical application. The model was subsequently validated with new data from the same center. RESULTS: A total of 196 JAMARs (97 JIA, 99 CMPS) were collected in the model development data, and 91 JAMARs (48 JIA, 43 CMPS) were collected in the validation data. Variables in the prediction model that were strongest associated with a diagnosis of JIA instead of CMPS were asymmetric pain/swelling in the shoulder (OR, 2.34), difficulty with self-care (OR, 2.41), skin rash (OR, 2.07), and asymmetric/pain swelling in the knee (OR, 2.29). Calibration and discrimination (area under the receiver operating characteristic curve, 0.83; 95% CI, 0.74-0.92) of the model in the validation data were good. CONCLUSIONS: Several items from the JAMAR questionnaire can potentially distinguish JIA from CMPS in patients with corresponding symptoms. We present an easy-to-use, adjusted, and validated model to separate these 2 diagnoses early at presentation based on patient-reported outcomes to facilitate proper referral and treatment.

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BACKGROUND: It is currently unknown whether patients with childhood-onset Takayasu disease (c-TA) are prone to physical inactivity and poor aerobic capacity. In this study, we assessed physical activity levels and cardiorespiratory fitness along with health-related quality of life (HRQL) and various traditional and non-traditional risk factors in patients with c-TA vs. healthy controls. METHODS: c-TA patients with non-active disease (n = 17) and age- and sex-matched healthy controls (n = 17) were enrolled in the study. We assessed physical activity levels, aerobic capacity, body composition, systemic inflammation, cardiometabolic markers, disease-related parameters, and HRQL. RESULTS: c-TA patients showed greater time spent in sedentary behavior (P = 0.010), and lower moderate-to-vigorous physical activity (P > 0.001) and lower step counts per day (P > 0.001). VO2peak (P < 0.001) and chronotropic response (P = 0.016) were significantly lower in patients with c-TA and they had worse HRQL in physical domain (P < 0.001), lower bone mineral content and density, and higher insulin levels vs. healthy controls (all P ≤ 0.05). CONCLUSIONS: c-TA patients exhibited reduced physical activity levels and aerobic capacity, worse cardiometabolic risk factors and HRQL parameter compared with healthy peers. Physical inactivity and aerobic deconditioning emerge as potentially novel risk factors for c-TA. The role of physical activity interventions in preventing poor outcomes and improving HRQL in c-TA remains to be explored.

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OBJECTIVES: To assess the level of resilience among patients with chronic musculoskeletal pain and their parents and to determine factors associated with patient and parental resilience. STUDY DESIGN: Cross-sectional cohort study of children aged 13-17 years diagnosed with chronic musculoskeletal pain and their parents. Patient-parent pairs were seen for initial consultation in the pediatric rheumatology pain clinic at Children's Hospital of Philadelphia between March and May 2018 and were administered a series of questionnaires including measures of resilience (Connor-Davidson Resilience Scale 10 item, The 14-item Resilience Scale, and the 7Cs of Resilience Tool). We calculated Pearson correlation coefficients to examine the relationship between the variables of interest and resilience. RESULTS: According to all resilience measures, patients and parents had low to moderate levels of resilience. These levels were lower than those previously reported among healthy populations, as well as those with chronic medical conditions. According to the Connor-Davidson Resilience Scale 10 item, patient-level resilience was negatively correlated with pain level (r = -0.48), physical disability (r = -0.54), and symptom severity (r = -0.53). The level of resilience among patients was positively correlated with energy level (r = 0.57) and health-related quality of life (r = 0.64). Parental resilience was positively correlated with parental mental health (r = 0.61). CONCLUSIONS: Higher patient resilience was correlated with reduced disease severity among adolescents with chronic musculoskeletal pain. Future research should explore whether fostering resilience in adolescents with chronic musculoskeletal pain via the application of resilience-training interventions mitigates disease burden in this vulnerable patient population.

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Determine disease activity in a low income juvenile idiopathic arthritis (JIA) cohort. 164 JIA patients from families with less than US$ 4500.00/capita mean annual income followed in Fortaleza-CE, Brazil, were cross-sectionally evaluated between May 2015-April 2016. Mean age was 14 ± 5.1 years (95 female) with 10.31 ± 3.7 years disease duration. Polyarticular category predominated, with 63 (38.4%) patients, followed by 40 (24%) enthesitis-related (ERA), and 36 (22%) oligoarticular. All but 1 out of 84 parents declared less than US$ 10,000.00 annual family income. Eighty-eight (60.7%) were receiving methotrexate and 19 (13%) leflunomide including 12 (63%) using both; 46 (28%) were on biologic DMARD including 20 (43.5%) adalimumab, 17 (41.5) etanercept, 5 (10.8%) tocilizumab, 2 (4.2%) abatacept, and 1 (2.1%) each on infliximab and canakinumab. Mean CHAQ and JADAS27 were 0.36 ± 0.55 and 5.31 ± 8.5, respectively. Thirty-two (20%) out of 159 patients had deformities. A bivariate analysis revealed that polyarticular had more deformities than oligoarticular patients (p = 0.002; OR = 2.389; 95% CI 1.37-4.14). Logistic regression showed no association between high JADAS and family income (p = 0.339; OR = 1.45; 95% CI 0.67-3.31). A general linear model showed significantly lower CHAQ score in patients from families earning more as compared to those earning less than 300.00 US$ monthly (p = 0.002). This study reports JIA disease activity in a low income population. Low income apparently did not influence prognosis given the low mean JADAS27 and CHAQ scores vis-à-vis data from other cohorts.

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Colombia is a densely populated country with a small number of pediatric rheumatology specialists, including 14 specialists for a population of 1,927,000 children in 2014. The objective of the study was to improve the skills required for early identification, timely referral, and management of musculoskeletal diseases, especially juvenile idiopathic arthritis (JIA), in a group of pediatricians and pediatric residents in a remote region of Colombia. Supported by grant programs developed by the International League of Associations for Rheumatology (ILAR), a combined educational strategy (blended learning) was implemented based on two classroom educational activities and four online modules. The students' acquired knowledge and perception of the strategy were evaluated. Scores were reported as median values and interquartile ranges (IQR), and the differences between scores were estimated using the Wilcoxon test for equal medians. Forty-one students were enrolled, 37 completed the online modules, and 33 attended the final in-person session. The results of the written tests demonstrated an improved ability to solve clinical problems compared with the results of the tests before the course (the median initial vs. final test scores 3 (IQR = 1) vs. 5 (IQR = 0), p = 0.000). The students reported high levels of satisfaction related to compliance with the proposed objectives, the relevance of the contents and activities performed, and the impact on everyday practice. These types of strategies are useful as tools for continuing medical education. However, the results pertain only to short-term learning. It is necessary to evaluate their impact on "lifelong learning."

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RESUMOObjetivoAvaliar as dimensões do fígado e do baço em pacientes com lúpus eritematoso sistêmico de início pediátrico (LESp) e controles saudáveis.MétodosForam submetidos a uma ultrassonografia do abdome 30 pacientes com LESp e 30 voluntários saudáveis controle. Foram feitas duas medições do fígado no lobo hepático esquerdo (craniocaudal e anteroposterior) e três no lobo hepático direito (LHD) (craniocaudal posterior [CCP-LHD], craniocaudal anterior e anteroposterior). Foram também avaliadas três medidas das dimensões do baço: longitudinal, transversal e anteroposterior. Foram avaliados dados demográficos, clínicos e laboratoriais, SLEDAI-2K, ECLAM, SLAM e tratamento.ResultadosA idade média foi semelhante nos pacientes com LESp e controles (170,31 ± 27,81 vs. 164,15 ± 39,25 meses; p = 0,486). A média da dimensão CCP-LHD foi significativamente maior no grupo LESp em comparação com os controles (13,30 ± 1,85 vs. 12,52 ± 0,93, p = 0,044). Não houve diferenças nos outros parâmetros biométricos do fígado e do baço (p > 0,05). Uma análise especifica realizada apenas nos pacientes com LESp de acordo com a dimensão CCP-LHD ≥ 13,3 cm versus < 13,3 cm mostrou que a mediana do SLEDAI-2K [8 (0-18) vs. 2 (0-8), p = 0,004], ECLAM [4 (0-9) vs. 2 (0-5), p = 0,019] e SLAM [5 (1-13) vs. 2 (0-14), p = 0,016] era significativamente maior em pacientes com maior dimensão CCP-LHD, do mesmo modo que a frequência de nefrite (77% vs. 29%, p = 0,010). As enzimas hepáticas foram semelhantes nos dois grupos (p > 0,05). Foi observada uma correlação positiva entre o SLEDAI-2K e a dimensão CCP-LHD (p = 0,001, r = +0,595). Evidenciou-se uma correlação negativa entre a duração da doença e a dimensão longitudinal do baço (p = 0,031, r = −0,394).ConclusãoOs dados levantam a possibilidade de que a atividade da doença pode levar a uma hepatomegalia subclínica e localizada durante o curso da doença. A duração da doença resultou em atrofia do baço em pacientes com LESp.

ABSTRACTObjectiveTo evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls.Methods30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed.ResultsMean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters (p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥ 13.3 cm versus < 13.3 cm showed that the median of SLEDAI-2K [8(0-18) vs. 2(0-8), p = 0.004], ECLAM [4(0-9) vs. 2(0-5), p = 0.019] and SLAM [5(1-13) vs. 2(0-14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups (p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL (p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen (p = 0.031, r = −0.394).ConclusionOur data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients.

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OBJECTIVE: To evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls. METHODS: 30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed. RESULTS: Mean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25 months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters (p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥ 13.3cm versus < 13.3 cm showed that the median of SLEDAI-2K [8(0-18) vs. 2(0-8), p=0.004], ECLAM [4(0-9) vs. 2(0-5), p = 0.019] and SLAM [5(1-13) vs. 2(0-14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups (p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL (p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen (p = 0.031, r = -0.394). CONCLUSION: Our data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients.

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La artritis idiopática juvenil (AIJ) ha sido definida por la Liga Internacional de Asociaciones de Reumatología (ILAR) como artritis de etiología desconocida que se inicia antes de los 16 años y dura por al menos seis semanas, habiendo excluido otras condiciones conocidas. La AIJ es una enfermedad cubierta por el sistema de Garantías Explícitas en Salud (GES) del Ministerio de Salud de Chile desde 2010. La presente guía, desarrollada por el Grupo Pediátrico de la Sociedad Chilena de Reumatología, consiste en una actualización de la Guía Clínica de AIJ 2010, incorporando nuevos protocolos terapéuticos y medicamentos que han demostrado un claro beneficio para niños con AIJ...

Juvenile idiopathic arthritis (JIA) has been defined by the International League of Associations for Rheumatology as arthritis of unknown etiology that begins before the sixteenth birthday and persists for at least 6 weeks with other known conditions excluded. JIA is a disease that is covered by the Explicit Health Guarantees system of the Chilean Ministry of Health since 2010. The present guideline developed by the Pediatric Group of the Chilean Rheumatology Society is an update of the 2010 JIA Clinical Guideline incorporating new treatment protocols and medications that have demonstrated clear benefits in children with JIA...

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