Percepção e enfrentamento da dor em crianças e adolescentes com fibromialgia juvenil e artrite idiopática juvenil poliarticular / Pain perception and pain coping mechanisms in children and adolescents with juvenile fibromyalgia and polyarticular juvenile idiopathic arthritis

RESUMO Objetivo: Mensurar e comparar a dor musculoesquelética em pacientes com fibromialgia juvenil (FMJ) e em pacientes com artrite idiopática juvenil poliarticular (AIJ); e avaliar e comparar a percepção e o enfrentamento da dor. Métodos: Foram avaliados, em estudo transversal, 150 crianças e adolescentes (e seus respectivos pais), divididos em três grupos: FMJ, AIJ e controles saudáveis. A mensuração e o enfrentamento da dor foram realizados por meio de instrumentos específicos. Para a avaliação da percepção da dor, desenvolveram-se três vinhetas com simulação de situações que pudessem gerar dor: aplicação de injeção, queda de bicicleta e isolamento social. Os pais e os pacientes responderam individualmente quanto à percepção da dor em cada situação. Resultados: As maiores notas de dor, os menores escores de enfrentamento da dor, as maiores notas para a percepção da dor nas vinhetas e os piores índices de qualidade de vida relacionada à saúde foram observados nos pacientes com FMJ, quando comparados aos pacientes com AIJ e aos controles. O mesmo padrão foi observado com os respectivos pais. Conclusões: Pacientes com AIJ e FMJ se comportam diferentemente em relação à percepção da dor e ao desenvolvimento de técnicas para o enfrentamento da dor. A dor deve ser avaliada sob diferentes perspectivas para um planejamento mais individualizado e efetivo do tratamento desses pacientes.

ABSTRACT Objective: To measure and compare musculoskeletal pain in patients with juvenile fibromyalgia (JFM) and polyarticular juvenile idiopathic arthritis (JIA), and to evaluate and compare pain perception and pain coping mechanisms in these patients. Methods: In this cross sectional study, we evaluated 150 children and adolescents, and their respective parents, from 3 different groups: JFM, polyarticular JIA, and healthy controls. Pain intensity and pain coping mechanisms were measured using specific questionnaires. Pain perception was evaluated according to three illustrations simulating situations that might cause pain: a shot, a bicycle fall, and social isolation. The patients' parents also filled out the questionnaires and provided a pain score that matched their child's perception of pain for each illustration. Results: The highest pain scores, the lowest pain coping strategy scores, the highest pain perception scores for all three illustrations, and the worse health related to quality of life indicators were observed in the JFM group, when compared to the JIA and control groups. The same pattern was observed with their parents. Conclusions: Patients with JIA and JFM behave differently in relation to pain perception and the development pain coping mechanisms. Pain should be evaluated from different perspectives for an individualized and efficient treatment of patients.

PAIN PERCEPTION AND PAIN COPING MECHANISMS IN CHILDREN AND ADOLESCENTS WITH JUVENILE FIBROMYALGIA AND POLYARTICULAR JUVENILE IDIOPATHIC ARTHRITIS. / PERCEPÇÃO E ENFRENTAMENTO DA DOR EM CRIANÇAS E ADOLESCENTES COM FIBROMIALGIA JUVENIL E ARTRITE IDIOPÁTICA JUVENIL POLIARTICULAR.

OBJECTIVE: To measure and compare musculoskeletal pain in patients with juvenile fibromyalgia (JFM) and polyarticular juvenile idiopathic arthritis (JIA), and to evaluate and compare pain perception and pain coping mechanisms in these patients. METHODS: In this cross sectional study, we evaluated 150 children and adolescents, and their respective parents, from 3 different groups: JFM, polyarticular JIA, and healthy controls. Pain intensity and pain coping mechanisms were measured using specific questionnaires. Pain perception was evaluated according to three illustrations simulating situations that might cause pain: a shot, a bicycle fall, and social isolation. The patients' parents also filled out the questionnaires and provided a pain score that matched their child's perception of pain for each illustration. RESULTS: The highest pain scores, the lowest pain coping strategy scores, the highest pain perception scores for all three illustrations, and the worse health related to quality of life indicators were observed in the JFM group, when compared to the JIA and control groups. The same pattern was observed with their parents. CONCLUSIONS: Patients with JIA and JFM behave differently in relation to pain perception and the development pain coping mechanisms. Pain should be evaluated from different perspectives for an individualized and efficient treatment of patients.

OBJETIVO: Mensurar e comparar a dor musculoesquelética em pacientes com fibromialgia juvenil (FMJ) e em pacientes com artrite idiopática juvenil poliarticular (AIJ); e avaliar e comparar a percepção e o enfrentamento da dor. MÉTODOS: Foram avaliados, em estudo transversal, 150 crianças e adolescentes (e seus respectivos pais), divididos em três grupos: FMJ, AIJ e controles saudáveis. A mensuração e o enfrentamento da dor foram realizados por meio de instrumentos específicos. Para a avaliação da percepção da dor, desenvolveram-se três vinhetas com simulação de situações que pudessem gerar dor: aplicação de injeção, queda de bicicleta e isolamento social. Os pais e os pacientes responderam individualmente quanto à percepção da dor em cada situação. RESULTADOS: As maiores notas de dor, os menores escores de enfrentamento da dor, as maiores notas para a percepção da dor nas vinhetas e os piores índices de qualidade de vida relacionada à saúde foram observados nos pacientes com FMJ, quando comparados aos pacientes com AIJ e aos controles. O mesmo padrão foi observado com os respectivos pais. CONCLUSÕES: Pacientes com AIJ e FMJ se comportam diferentemente em relação à percepção da dor e ao desenvolvimento de técnicas para o enfrentamento da dor. A dor deve ser avaliada sob diferentes perspectivas para um planejamento mais individualizado e efetivo do tratamento desses pacientes.

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterised by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However, approximately half of children with sJIA develop destructive, long-standing arthritis that appears similar to other forms of JIA. Using genomic approaches, we sought to gain novel insights into the pathophysiology of sJIA and its relationship with other forms of JIA. METHODS: We performed a genome-wide association study of 770 children with sJIA collected in nine countries by the International Childhood Arthritis Genetics Consortium. Single nucleotide polymorphisms were tested for association with sJIA. Weighted genetic risk scores were used to compare the genetic architecture of sJIA with other JIA subtypes. RESULTS: The major histocompatibility complex locus and a locus on chromosome 1 each showed association with sJIA exceeding the threshold for genome-wide significance, while 23 other novel loci were suggestive of association with sJIA. Using a combination of genetic and statistical approaches, we found no evidence of shared genetic architecture between sJIA and other common JIA subtypes. CONCLUSIONS: The lack of shared genetic risk factors between sJIA and other JIA subtypes supports the hypothesis that sJIA is a unique disease process and argues for a different classification framework. Research to improve sJIA therapy should target its unique genetics and specific pathophysiological pathways.

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features.

OBJECTIVE: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. METHODS: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit. RESULTS: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (±4.2) years and 1.2 (±1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. CONCLUSION: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features / Estudo multicêntrico brasileiro de 71 pacientes com arterite de Takayasu juvenil: características clínicas e angiográficas

ABSTRACT Objective: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. Methods: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6 th to 12th month of diagnosis, and in the last visit. Results: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (± 4.2) years and 1.2 (± 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. Conclusion: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.

RESUMO Objetivo: Descrever as características clínicas e angiográficas da arterite de Takayasu em crianças e adolescentes brasileiros. Métodos: Foi feita coleta retrospectiva de dados de 71 crianças e adolescentes acompanhados em 10 centros brasileiros de referência em reumatologia pediátrica. A avaliação foi feita em três tempos: início dos sintomas até o diagnóstico, do 6º ao 12º mês de diagnóstico e última consulta. Resultados: Dos 71 pacientes selecionados, 51 (71,8%) eram meninas. As médias de idade de início dos sintomas e de tempo até diagnóstico foram 9,2 anos (± 4,2) e 1,2 anos (± 1,4), respectivamente. No fim do estudo, 20 pacientes estavam em atividade de doença, 39 em remissão e cinco haviam evoluído a óbito. Os sintomas mais frequentes nas avaliação inicial, segunda avaliação e avaliação final foram, respectivamente, os constitucionais, os musculoesqueléticos e os neurológicos. A redução de pulsos periféricos foi o sinal cardiovascular mais frequente e a elevação da velocidade de hemossedimentação foi o achado laboratorial mais frequente nos três períodos de avaliação. O teste tuberculínico foi reagente em 41%. A estenose foi a lesão angiográfica mais encontrada, a artéria abdominal foi o segmento mais afetado e tipo angiográfico IV o mais frequente. A maioria (90%) fez terapia com glicocorticoides, 85,9% necessitaram de outro imunossupressor e 29,6% foram submetidos à angioplastia. Conclusão: Este é o maior estudo de arterite de Takayasu juvenil e nós observar elevado número de pacientes com idade inferior a 10 anos e a predominância de sintomas constitucionais no início da doença.

HLA-DRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis.

Systemic juvenile idiopathic arthritis (sJIA) is an often severe, potentially life-threatening childhood inflammatory disease, the pathophysiology of which is poorly understood. To determine whether genetic variation within the MHC locus on chromosome 6 influences sJIA susceptibility, we performed an association study of 982 children with sJIA and 8,010 healthy control subjects from nine countries. Using meta-analysis of directly observed and imputed SNP genotypes and imputed classic HLA types, we identified the MHC locus as a bona fide susceptibility locus with effects on sJIA risk that transcended geographically defined strata. The strongest sJIA-associated SNP, rs151043342 [P = 2.8 × 10(-17), odds ratio (OR) 2.6 (2.1, 3.3)], was part of a cluster of 482 sJIA-associated SNPs that spanned a 400-kb region and included the class II HLA region. Conditional analysis controlling for the effect of rs151043342 found that rs12722051 independently influenced sJIA risk [P = 1.0 × 10(-5), OR 0.7 (0.6, 0.8)]. Meta-analysis of imputed classic HLA-type associations in six study populations of Western European ancestry revealed that HLA-DRB1*11 and its defining amino acid residue, glutamate 58, were strongly associated with sJIA [P = 2.7 × 10(-16), OR 2.3 (1.9, 2.8)], as was the HLA-DRB1*11-HLA-DQA1*05-HLA-DQB1*03 haplotype [6.4 × 10(-17), OR 2.3 (1.9, 2.9)]. By examining the MHC locus in the largest collection of sJIA patients assembled to date, this study solidifies the relationship between the class II HLA region and sJIA, implicating adaptive immune molecules in the pathogenesis of sJIA.

Are temporomandibular joint signs and symptoms associated with magnetic resonance imaging findings in juvenile idiopathic arthritis patients? A longitudinal study.

The aims of this longitudinal study were to perform a comprehensive clinical evaluation of temporomandibular joint (TMJ) and to investigate the association between the clinical and magnetic resonance imaging (MRI) findings in the TMJs of patients with juvenile idiopathic arthritis (JIA). Seventy-five patients with JIA participated in this study. All patients underwent a rheumatological examination performed by a paediatric rheumatologist, a TMJ examination performed by a single dentist and an MRI with contrast of the TMJs. These examinations were scheduled on the same date. The patients were examined again 1 year later. Twenty-eight (37.3 %) patients reported symptoms at the first evaluation and 11 (14.7 %) patients at the second evaluation. In relation to signs, 35 (46.7 %) of the patients presented at least one sign at the first evaluation and 29 (38.7 %) at the second. Intense contrast enhancement of TMJ was significantly associated with disease activity (p < 0.001) at the first evaluation and a trend to significance was observed at the second (p = 0.056), with poly/systemic subtypes (p = 0.028 and p = 0.049, respectively), with restricted mouth opening capacity (p = 0.013 and p = 0.001, respectively), with the presence of erosions at both evaluations (p = 0.0001 and p < 0.0001, respectively) and with altered condylar shape at the second evaluation (p = 0.0005). TMJ involvement is highly prevalent in JIA patients, with asymptomatic children presenting severe structural alterations of the TMJ. The TMJ should always be evaluated in JIA patients, even in the absence of signs and symptoms.

Fatigue in children with juvenile idiopathic arthritis: reliability of the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale".

The aim of the study was (1) to translate the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale" (PedsQL-Fatigue) into Brazilian Portuguese language and culture and evaluate its reliability and (2) to measure fatigue among patients with juvenile idiopathic arthritis (JIA): (1) Translation of the PedsQL-Fatigue by two bilingual researchers; (2) Backtranslation into English assessed by the authors of the original version; (3) Pilot study with five patients followed in the Pediatric Rheumatology Outpatient Clinic and their parents; and (4) Field study and assessment of measurement properties (internal consistency, reproducibility, and construct validity). In this stage, the scale was administered to 67 patients with JIA and 63 healthy individuals, aged from 2 to 18 years old, matched by age (from 2 to 4, 5 to 7, 8 to 12, and from 13 to 18 years old). Cronbach's alpha coefficient ranged from 0.6 to 0.8 for children and parents, indicating the instrument's good internal consistency. The scale's construct validity was confirmed by a satisfactory Spearman's coefficient between the PedsQL-Fatigue and the generic PedsQL 4.0 (0.840 for the children and 0.742 for the parents). Reproducibility was also adequate (0.764 for the children and 0.938 for the parents). No differences were found between the scores obtained by the JIA group and control group, though lower scores were observed among patients with clinically active JIA when compared to those without clinical activity. The PedsQL-Fatigue is a valid and reliable tool, and that can be used to measure fatigue among patients with JIA.

Do patients with juvenile idiopathic arthritis in remission exhibit active synovitis on joint ultrasound?

The aim of the study was to assess the presence and characteristics of subclinical synovitis using power Doppler (PD) ultrasonography on patients with juvenile idiopathic arthritis (JIA) in clinical remission and compare the findings with those of healthy children. A cross-sectional study was carried out involving the clinical (physical exam, functional capacity and laboratory tests) and ultrasonography evaluation of 34 joints (synovial fluid/hypertrophy, PD signal and bone erosion). Subclinical synovitis was defined as the presence of synovial hypertrophy/joint effusion with or without any PD signal. Thirty-six patients (11.5 ± 3.74 years) and 36 controls (sex and age matched) were evaluated (2,448 joints). Twenty-seven patients were in remission on medication (mean duration: 1.8 ± 2.2 years). Subclinical synovitis was detected in 41.7% patients and 11.1% controls (p = 0.003). Erosion was detected in three patients (8.3%). Subclinical synovitis was found in 38/1,224 (3.1%) joints in the patients (most affected: radiocarpal wrist, anterior elbow and tibiotalar ankle) and 8/1,224 (0.6%) joints in the controls (most affected: radiocarpal wrist). Differences in subclinical synovitis between patients and controls were found in the elbows (p = 0.033) and ankles (p = 0.006). A greater frequency of subclinical synovitis was found in patients with the extended oligoarticular or polyarticular subtypes (p = 0.013), those at an older age at disease onset (p = 0.007) and using methotrexate (p = 0.049). Patients with JIA in remission exhibit subclinical synovitis more frequently than controls. Subclinical synovitis was more frequent in patients with the polyarticular involvement and those at an older age at disease onset.

Health related quality of life of children with rheumatic heart diseases: reliability of the Brazilian version of the Pediatric Quality of Life Inventory™ Cardiac Module scale.

BACKGROUND: This study aimed to translate the 'Pediatric Quality of Life Inventory™ (PedsQL™ 3.0) Cardiac Module' into Portuguese, adapt it to Brazilian culture, and assess its psychometric properties (validity and reproducibility), and to calculate health-related quality of life scores on the PedsQL 4.0 and PedsQL™ 3.0 Cardiac Module Scales for a group of patients 5 to 18 years old with rheumatic heart disease. METHODS: The methods suggested by the authors of the original version of the questionnaire included 1) translation by an expert panel; 2) translation back into English and revision by the authors of the original version; 3) pilot study with seven children and parents in each of three age ranges (5 to 7, 8 to 12, and 13 to 18 years old); and 4) assessment of the measurement properties. In this stage, the PedsQL™ 3.0 Cardiac Module and the PedsQL 4.0 Generic Scale were applied to a sample comprising 109 children and adolescents with rheumatic heart disease and their parents or caregivers. The version for parents or caregivers was administered separately on the same day. RESULTS: The values of Cronbach's alpha for all scales assessed in the questionnaire (heart problems and treatment [symptoms], problems with perceived physical appearance, treatment anxiety, cognitive problems, and communication problems) varied from 0.6 to 0.8, indicating good internal consistency. Correlation was found between the scores for the Cardiac Module and the Generic Scale (0.36-0.86), demonstrating convergent validity (Spearman's correlation coefficient, p < 0.01). The symptoms, problems with perceived physical appearance, and cognitive and communication problem domains were able to distinguish between groups of patients with mild and moderate/severe heart disease (Student's t-test, p < 0.05). The intraclass correlation of the interobserver reproducibility was adequate (0.76 to 0.94 among the patients [children/adolescents] and 0.76 to 0.84 among their caregivers). The correlation between the patients' scores and their parents' scores varied from 0.50 to 0.86 (Pearson's correlation coefficient, p < 0.01). CONCLUSIONS: The Brazilian version of the PedsQL™ 3.0 Cardiac Module was shown to be reliable. The application of this questionnaire in practice will be very useful for all professionals charged with the care of children and adolescents with heart diseases.

Association between the ultrasonographic and clinical findings in the hips of patients with juvenile idiopathic arthritis.

OBJECTIVE: To describe the ultrasonographic (US) findings in the hips of patients with juvenile idiopathic arthritis (JIA) and the association between these findings and the signs, symptoms, and activity of the disease. MATERIALS AND METHODS: The present retrospective study included 92 patients with JIA. The disease subtypes, age at disease onset, length of disease progression, disease activity, and clinical manifestations of the hip pathology were assessed. US examinations were routinely performed, and the images were analysed by two ultrasonographers who were blinded to the patients' clinical conditions. RESULTS: Of the 92 patients included in the study, 69.6% were girls, and the average age was 12.4 ± 5.1 years. Thirty-three (35.9%) participants exhibited the persistent oligoarticular subtype, and 30 (32.6%) exhibited the rheumatoid factor (RF)-negative polyarticular subtype. Forty-four participants exhibited signs and/or symptoms of hip pathology. Twenty-nine (31.5%) participants exhibited abnormal US findings, and 34.4% exhibited subclinical synovitis. The US alterations exhibited an association with subclinical synovitis in 34.4% of the cases. The US alterations bore a correlation with the presence of hip-related signs and/ or symptoms (P = 0.021), particularly joint limitations (P = 0.006), but were not correlated with the disease activity (P = 0.948) or subtype (P = 0.108). Clinical synovitis was associated with polyarticular involvement (P = 0.002) and disease activity (P = 0.017). Subclinical synovitis was not correlated with the investigated variables. CONCLUSION: Clinical affection of the hip in JIA, particularly joint limitation, is associated with synovitis (revealed by US assessment) independently of the activity and subtype of the disease. Therefore, healthcare professionals should consider the possible occurrence of silent disease and subclinical synovitis, which might contribute to hip deterioration.

Dyslipidemia in pediatric systemic lupus erythematosus: the relationship with disease activity and plasma homocysteine and cysteine concentrations.

OBJECTIVE: To evaluate the presence of dyslipidemia and plasma concentrations of homocysteine (Hcy) and cysteine (Cys) in adolescents with juvenile systemic lupus erythematosus (SLE) and relate these findings to disease activity (Systemic Lupus Erythematosus Disease Activity Index, SLEDAI) and cardiovascular risk factors. METHODS: A cross-sectional controlled study including 26 female adolescents with SLE and 26 healthy controls was conducted. We evaluated SLEDAI, medications, anthropometric data, dietary intake, lipid profile, proteinuria, Hcy, Cys, folic acid, vitamin B12, and high-sensitivity C-reactive protein levels. RESULTS: Dyslipidemia was observed in 46.2% of the patients and in 19.2% of the controls. The SLE group had a higher Cys concentration and a lower high-density lipoprotein cholesterol concentration compared with the controls. In the multivariate analysis only Hcy was significantly and independently associated with the presence of dyslipidemia in the juvenile SLE group; an increase of 1 µmol/l in the Hcy concentration doubled the chance of dyslipidemia (OR: 2.1; 95% CI: 1.1-4.9; p = 0.030). The Cys concentration was correlated with Hcy, total cholesterol, low-density lipoprotein cholesterol, and triglyceride concentrations. CONCLUSION: We observed the presence of cardiovascular risk factors in adolescents with juvenile SLE. The early identification of biochemical alterations allows the development of intervention strategies that may lower the risk of cardiovascular disease.

Associação entre achados ultrassonográficos e clínicos do quadril de pacientes com artrite idiopática juvenil / Association between the ultrasonographic and clinical findings in the hips of patients with juvenile idiopathic arthritis

OBJETIVO: Descrever os achados ultrassonográficos do quadril em pacientes com artrite idiopática juvenil (AIJ) e sua associação com sinais, sintomas e atividade da doença. MATERIAIS E MÉTODOS: Estudo retrospectivo com 92 pacientes com AIJ. Foram avaliados os subtipos da doença, a idade de início, o tempo de evolução, a atividade da doença e as manifestações clínicas do envolvimento do quadril. A avaliação ultrassonográfica foi realizada na rotina dos pacientes, e as imagens foram analisadas por dois ultrassonografistas cegos em relação às condições clínicas dos pacientes. RESULTADOS: Do total de 92 pacientes, 69,6% eram meninas, com média de idade de 12,4 ± 5,1 anos. Trinta e três (35,9%) apresentaram subtipo oligoarticular persistente e trinta (32,6%) poliarticular com fator reumatoide negativo. Quarenta e quatro (47,8%) apresentaram sinal e/ou sintoma relacionado ao quadril. Vinte e nove (31,5%) apresentaram alterações ultrassonográficas, com sinovite subclínica em 34,4%. As alterações ultrassonográficas se associaram com presença de sinais e/ou sintomas do quadril (P = 0,021), especialmente limitação articular (P = 0,006), mas não com atividade (P = 0,948) ou subtipo de doença (P = 0,108). Sinovite clínica se associou com comprometimento poliarticular (P = 0,002) e atividade de doença (P = 0,017). Não houve associação entre sinovite subclínica e as variáveis estudadas. CONCLUSÃO: O acometimento clínico do quadril na AIJ, especialmente a limitação articular, está associado à sinovite na avaliação por US, independente do subtipo e da atividade da doença. Os profissionais de saúde devem estar atentos à possibilidade de doença silenciosa com sinovite subclínica, que pode contribuir para a deterioração da articulação do quadril.

OBJECTIVE: To describe the ultrasonographic (US) findings in the hips of patients with juvenile idiopathic arthritis (JIA) and the association between these findings and the signs, symptoms, and activity of the disease. MATERIALS AND METHODS: The present retrospective study included 92 patients with JIA. The disease subtypes, age at disease onset, length of disease progression, disease activity, and clinical manifestations of the hip pathology were assessed. US examinations were routinely performed, and the images were analysed by two ultrasonographers who were blinded to the patients' clinical conditions. RESULTS: Of the 92 patients included in the study, 69.6% were girls, and the average age was 12.4 ± 5.1 years. Thirty-three (35.9%) participants exhibited the persistent oligoarticular subtype, and 30 (32.6%) exhibited the rheumatoid factor (RF)-negative polyarticular subtype. Forty-four participants exhibited signs and/or symptoms of hip pathology. Twentynine (31.5%) participants exhibited abnormal US findings, and 34.4% exhibited subclinical synovitis. The US alterations exhibited an association with subclinical synovitis in 34.4% of the cases. The US alterations bore a correlation with the presence of hip-related signs and/ or symptoms (P = 0.021), particularly joint limitations (P = 0.006), but were not correlated with the disease activity (P = 0.948) or subtype (P = 0.108). Clinical synovitis was associated with polyarticular involvement (P = 0.002) and disease activity (P = 0.017). Subclinical synovitis was not correlated with the investigated variables. CONCLUSION: Clinical affection of the hip in JIA, particularly joint limitation, is associated with synovitis (revealed by US assessment) independently of the activity and subtype of the disease. Therefore, healthcare professionals should consider the possible occurrence of silent disease and subclinical synovitis, which might contribute to hip deterioration.

Quality of life in patients with sickle cell disease.

OBJECTIVE: To evaluate the quality of life in children and adolescents with sickle cell disease attending a blood reference center, and to assess the quality of life of their relatives. METHODS: Cross-sectional study that included 100 patients with sickle cell disease, which were divided into three subgroups according to age: 5 to 7 (n=18), 8 to 12 (n=32), and 13 to 18 years-old (n=50), and their parents. The Control Group included 50 healthy children and adolescents from a public local school, also divided into the same three age subgroups and their caregivers. The Pediatric Quality of life Inventory (PedsQL), version 4.0, was applied in both groups. The generic questionnaire Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36) was applied to the relatives. The answers were linearly transformed into a score and compared by non-parametric tests. RESULTS: The PedsQL scores of patients were significantly lower than those obtained in the Control Group (p<0.0001) in all studied areas (physical, emotional, social skills, and school activities). Similarly, SF-36 scores applied to the patients' parents were lower than those obtained in the Control Group in all studied aspects (p<0.0001). CONCLUSIONS: Sickle cell disease affects the quality of life of children, adolescents, and their families. Patients sense restrictions in the emotional, social, family and physical aspects, among others.

Qualidade de vida em portadores de doença falciforme / Quality of life in patients with sickle cell disease / Calidad de vida en portadores de enfermedad falciforme

OBJETIVO: Avaliar a qualidade de vida relacionada à saúde de crianças e adolescentes com doença falciforme assistidas em um hemocentro de referência e mensurar a qualidade de vida relacionada à saúde dos respectivos familiares. MÉTODOS: Estudo transversal e seccional com 100 pacientes portadores de doença falciforme, divididos em três subgrupos conforme a faixa etária: de 5 a 7 (n=18), de 8 a 12 (n=32) e de 13 a 18 anos (n=50) e com seus respectivos pais. O Grupo Controle foi composto por 50 crianças e adolescentes saudáveis de uma escola pública local, também divididos nos três subgrupos de idade e seus respectivos cuidadores. Foi aplicado o questionário genérico "Pediatric Quality of Life Inventory" (PedsQL), versão 4.0, em ambos os grupos. Aos familiares foi aplicado o questionário genérico Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36). As respostas obtidas foram linearmente transformadas em um escore e comparadas com o auxílio de testes não paramétricos. RESULTADOS: Os escores dos pacientes no PedsQL foram inferiores àqueles do Grupo Controle (p<0,0001) nos aspectos estudados (capacidades física, emocional, social e atividade escolar). Da mesma forma, os escores do SF-36 aplicados aos pais dos pacientes foram mais baixos que os de pais do Grupo Controle em todos os aspectos estudados (p<0,0001). CONCLUSÕES: A doença falciforme compromete a qualidade de vida das crianças, dos adolescentes e de suas respectivas famílias. Os pacientes percebem restrições nos aspectos emocional, social, familiar e físico, dentre outros.

OBJECTIVE: To evaluate the quality of life in children and adolescents with sickle cell disease attending a blood reference center, and to assess the quality of life of their relatives. METHODS: Cross-sectional study that included 100 patients with sickle cell disease, which were divided into three subgroups according to age: 5 to 7 (n=18), 8 to 12 (n=32), and 13 to 18 years-old (n=50), and their parents. The Control Group included 50 healthy children and adolescents from a public local school, also divided into the same three age subgroups and their caregivers. The Pediatric Quality of life Inventory (PedsQL), version 4.0, was applied in both groups. The generic questionnaire Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36) was applied to the relatives. The answers were linearly transformed into a score and compared by non-parametric tests. RESULTS: The PedsQL scores of patients were significantly lower than those obtained in the Control Group (p<0.0001) in all studied areas (physical, emotional, social skills, and school activities). Similarly, SF-36 scores applied to the patients' parents were lower than those obtained in the Control Group in all studied aspects (p<0.0001). CONCLUSIONS: Sickle cell disease affects the quality of life of children, adolescents, and their families. Patients sense restrictions in the emotional, social, family and physical aspects, among others.

OBJETIVO: Evaluar la calidad de vida relacionada a la salud en niños y adolescentes con enfermedad falciforme asistidas en un servicio de hemoterapia de referencia y medir la calidad de vida relacionada a la salud de los respectivos familiares. MÉTODOS: Estudio transversal y seccional en 100 pacientes portadores de enfermedad falciforme, divididos en tres subgrupos conforme a la franja de edad: de 5 a 7 (n=18), de 8 a 12 (n=32) y de 13 a 18 (n=50) años con sus respectivos padres. El Grupo Control fue compuesto por 50 niños y adolescentes sanos de una escuela pública local, también divididos en los mismos tres subgrupos de edad y sus respectivos cuidadores. Se aplicó el cuestionario genérico «Pediatric Quality of Life Inventory¼ (PedsQL), versión 4.0, a ambos grupos. A los familiares se aplicó el cuestionario genérico Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36). Las respuestas obtenidas fueron linealmente transformadas en un escore y comparadas con la ayuda de pruebas no paramétricas. RESULTADOS: Los escores de los pacientes en el PedsQL fueron inferiores a aquellos del Grupo Control (p<0,0001) en los aspectos estudiados (capacidades física, emocional, social y actividad escolar). Del mismo modo, los escores del SF-36 aplicados a los padres de los pacientes fueron más bajos que los de padres del Grupo Control en todos los aspectos estudiados (p<0,0001). CONCLUSIONES: La enfermedad falciforme compromete la calidad de vida de los niños, de los adolescentes y de sus respectivas familias. Los pacientes perciben restricciones en los aspectos emocional, social, familiar y físico, entre otros.

Prevalence of juvenile idiopathic arthritis in children aged 6 to 12 years in Embu das Artes, state of Sao Paulo, Brazil.

The aim of the study was to study the prevalence of juvenile idiopathic arthritis (JIA) in school children in the city of Embu das Artes in São Paulo State. 2880 school children from seven public schools, aged between 6 and 12 years, were evaluated (clinical findings) by a pediatric rheumatologist. A board certified Pediatric Rheumatologist evaluated the subjects with suspected inflammatory arthropathy. Children with higher suspicion were referred to a specialized service. One hundred and forty-one children have presented abnormalities on examination of musculoskeletal system, with isolated pain on palpation the most common finding in the first evaluation (60.9%), with improvement in almost all cases in the second examination. Most of the abnormalities were related to recent injuries or congenital malformations. Six children have clinical findings suggestive of chronic arthropathy and were referred to a specialized pediatric rheumatology clinic. Of these, a 12 year-old girl fulfilled the criteria for JIA. The other diagnoses were aseptic necrosis of the hip (P = 1) of and post-trauma synovitis (P = 4). The prevalence of JIA in children aged between 6 and 12 years was 1/2.880 (or 0.34/1.000).

Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases.

OBJECTIVE: There are several factors that contribute to poor adherence to treatment in children and adolescents with chronic rheumatic diseases, worsening their quality of life and prognosis. Our aim was to assess the rates of adherence to treatment and to identify the socioeconomic and clinical factors associated. METHODS: The sample included 99 patients with juvenile idiopathic arthritis, systemic erythematosus lupus, dermatomyositis or juvenile scleroderma. All patients were followed at the outpatient pediatric rheumatology for a minimum period of 6 months. To assess adherence, a questionnaire was administered to the providers, which included three blocks: 1) demographic, clinical and laboratory data; 2) medication adherence; and 3) attending follow-up appointments, examinations and use of orthoses. A value lower than or equal to 80% of the prescribed was considered poor adherence. RESULTS: A total of 53% of patients showed good overall adherence, observed when the caregiver lived in a stable union marital status (p = 0.006); 20 patients (20.2%) presented poor medication adherence, related to the use of three or more medications daily (p = 0.047). The causes of poor adherence were forgetfulness, refusal, incorrect dose or lack of medication, personal problems, and financial difficulties. CONCLUSIONS: We observed good overall treatment adherence in patients whose providers lived in stable union and poor adherence to medication in patients who used more than three types of medication daily. There was no association between the adherence rates and sex, age, time since diagnosis and disease activity.

Fatores associados à adesão ao tratamento de crianças e adolescentes com doenças reumáticas crônicas / Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases

OBJETIVO: São vários os fatores que contribuem para a má adesão ao tratamento de crianças e adolescentes com doenças reumáticas crônicas, gerando piora da qualidade de vida e do prognóstico. Nosso objetivo foi avaliar as taxas de adesão ao tratamento e identificar os fatores socioeconômicos e clínicos associados. MÉTODOS: Foram incluídos 99 pacientes com artrite idiopática juvenil, lúpus eritematoso sistêmico, dermatomiosite ou esclerodermia juvenil. Todos os pacientes eram acompanhados no ambulatório de reumatologia pediátrica por um período mínimo de 6 meses. Para avaliação da adesão, foi aplicado aos cuidadores um questionário composto por três blocos: 1) dados demográficos, clínicos e laboratoriais; 2) adesão ao tratamento medicamentoso; e 3) comparecimento às consultas, realização de exames e utilização de órteses. Foi considerada má adesão, quando realizado valor menor ou igual a 80% do prescrito. RESULTADOS: Um total de 53% dos pacientes apresentou boa adesão ao tratamento global, observada quando o cuidador possuía união estável (p = 0,006); 20 pacientes (20,2%) apresentaram má adesão ao tratamento medicamentoso, relacionada à utilização de mais que três medicamentos diários (p = 0,047). As causas de má adesão ao tratamento foram esquecimento, recusa, dose incorreta ou falta de medicamento, problemas pessoais e dificuldades financeiras. CONCLUSÕES: Observamos boa adesão ao tratamento global nos pacientes cujos cuidadores possuíam união estável e má adesão ao tratamento medicamentoso nos pacientes que utilizavam mais que três tipos de medicamentos diariamente. Não houve associação entre as taxas de adesão ao tratamento e sexo, idade, tempo de diagnóstico e atividade da doença.

OBJECTIVE: There are several factors that contribute to poor adherence to treatment in children and adolescents with chronic rheumatic diseases, worsening their quality of life and prognosis. Our aim was to assess the rates of adherence to treatment and to identify the socioeconomic and clinical factors associated. METHODS: The sample included 99 patients with juvenile idiopathic arthritis, systemic erythematosus lupus, dermatomyositis or juvenile scleroderma. All patients were followed at the outpatient pediatric rheumatology for a minimum period of 6 months. To assess adherence, a questionnaire was administered to the providers, which included three blocks: 1) demographic, clinical and laboratory data; 2) medication adherence; and 3) attending follow-up appointments, examinations and use of orthoses. A value lower than or equal to 80% of the prescribed was considered poor adherence. RESULTS: A total of 53% of patients showed good overall adherence, observed when the caregiver lived in a stable union marital status (p = 0.006); 20 patients (20.2%) presented poor medication adherence, related to the use of three or more medications daily (p = 0.047). The causes of poor adherence were forgetfulness, refusal, incorrect dose or lack of medication, personal problems, and financial difficulties. CONCLUSIONS: We observed good overall treatment adherence in patients whose providers lived in stable union and poor adherence to medication in patients who used more than three types of medication daily. There was no association between the adherence rates and sex, age, time since diagnosis and disease activity.