The musculoskeletal impairment negatively impacts the quality of life of children and adolescents with psoriasis

Abstract Background The severity of nail disease, the presence of arthralgia and fatigue are predictors of development of psoriatic arthritis (PsA) in patients with psoriasis (Pso). In children, little is known about the musculoskeletal (MSK) impairment in patients with Pso and its effect on health-related quality of life (HRQoL). Objectives To determine the frequencies of pain and MSK inflammation (i.e., arthritis, enthesitis, and sacroiliitis) among children and adolescents with Pso and its relationship to HRQoL and fatigue. Methods Pediatric patients with Pso underwent a rheumatologic physical examination to evaluate synovitis, enthesalgia, sacroiliac joint (SIJ) pain and tender points of fibromyalgia. The core set of domains recommended by the GRAPPA - OMERACT to be measured in PsA studies was assessed. Ultrasound (US) was performed in clinical cases of enthesitis, and magnetic resonance imaging (MRI) was performed in cases of SIJ pain. Results Forty-three participants (10 ± 2.9 years old) were evaluated. Pain on palpation of the entheses was observed in 10 (23.2%) patients and pain on SIJ palpation was observed in 3 (7%). No patient presented with synovitis; one presented with enthesitis on US, but MRI did not confirm sacroiliitis in any case. Patients with MSK pain had greater skin disease severity (PASI 5.4 vs. 2, p < 0.01), worse fatigue, and lower HRQoL scores on all instruments used. The estimated risk of HRQoL impairment was eight times higher in the presence of MSK pain, which was an independent predictive factor. With a NAPSI greater than 30, the probability of pain was greater than 80%. Conclusion MSK pain is frequent among children with Pso, related to the severity of skin and nail disease, and negatively affects HRQoL. The typically used complementary exams might not detect the inflammatory process caused by Pso.(AU)

Adolescents social needs living with juvenile idiopathic arthritis and their views about digital resources

Abstract Background: Juvenile Idiopathic Arthritis (JIA) and its related symptoms (e.g. pain) have been associated with interference in the daily life of adolescents with JIA including their friendships. There is little research in that sense and in consequence, interventions designed to improve this area. The objectives of this study are 1) to gain knowledge about the needs of adolescents with JIA, particularly focused on their friendships; 2) to explore the potential of the Internet to help them, and 3) to determine what kind of online resource would be the best and what elements it should include. Methods: To achieve the proposed objectives we designed a qualitative study including two phases: the first one exploratory (semi-structured interviews) and the following, confirmatory (online focus group). Results: 14 adolescents were interviewed and 7 participated in the focus group. They reported some social challenges related to their illness: feeling different, criticized by peers, or not believed. Additionally, they specified some of the coping strategies they used, such as disclosing to others that they have JIA, using communication skills, maintaining activities with friends, trying to minimize pain, and ignoring negative comments. Adolescents considered an online resource useful and mentioned that they would like to find general information and to have the possibility to interact with others. They considered Instagram and WhatsApp as good platforms to implement the online resource. Conclusions: According to their perceptions, adolescents with JIA can benefit from an online resource which delivers information, strategies and facilitates interaction with others.(AU)

Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

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.

Prevalence and factors of functional disability in patients with juvenile idiopathic arthritis

The aim of this study was to identify the prevalence of functional disability and the possible factors that may be associated with functional disability in children and adolescents with juvenile idiopathic arthritis (JIA) in Sharkia Governorate. Methods: This cross sectional study was carried out in Rheumatology and Rehabilitation department, Faculty of Medicine, Zagazig University Hospitals. Forty-eight consecutive patients of JIA aged from 7 to 17 years underwent assessment of socio-economic and demographic characteristics, functional ability using the childhood health assessment questionnaire (CHAQ), disease activity using the Juvenile Arthritis Disease Activity Score based on 27 joints (JADAS-27), psychological symptoms using the Children's Depression Inventory (CDI) score. Results: Multivariate modeling was applied to determine the factors that associated with functional disability. A total of 80 % of the patients (39 of 48) had functional disability. In multiple regression analyses, high CDIscores (OR 20.78, 95 % CI 1.64 to 262.91, P =.019),JADAS-27 (OR 17.49% CI 2.16 to 141.62, P =.007), low socioeconomic status (OR 10.43, 95 % CI 1.24 to 87.57, P =.031) were strong predictors of functional disability in JIA patients.Conclusion: Our study on patients with JIA provides evidence suggesting that a total of 80 % of the patients had functional disability, that is associated with higher CDI scores, higher JADAS -27 and a low socioeconomic status

Lower genital tract infections in young female juvenile idiopathic arthritis patients

Abstract Background: To evaluate human papillomavirus (HPV), Chlamydia trachomatis (CT) and Neisseria gonorrhoeae (NG) infections in juvenile idiopathic arthritis (JIA) patients. Methods: After exclusion, 33 female adolescent and young JIA patients (ILAR criteria) and 28 healthy controls were selected for this study. Demographic data, gynecological, sexual function, cervical cytology and histological abnormalities were evaluated. JIA clinical/laboratorial parameters and treatment were also assessed. HPV-DNA, CT-DNA and NG-DNA testing in cervical specimens were performed by Hybrid Capture 2 assays. Results: The mean current age was similar in JIA patients and controls (23.3 ± 6.24 vs. 26.1 ± 6.03 years, p = 0.09). The frequencies of sexual intercourse (76% vs. 89%, p = 0.201) and abnormal cervical cytology (24% vs. 11%, p = 0.201) were similar in JIA compared to controls. The higher frequency of HPV infection in JIA patients than controls (30% vs. 11%, p = 0.155) did not reach statistical significance. CT (0% vs. 7%, p = 0.207) and NG infections (0% vs. 4%, p = 0.459) were also alike in both groups. Further evaluation of JIA patients with abnormal and normal cervical cytology showed that the former group had a higher frequency of HPV infection (87% vs. 12%, p = 0.0002) with a low frequency of HPV vaccination (0% vs. 8%, p = 1.0). No differences were evidenced between these two JIA groups regarding demographic data, sexual function and clinical/laboratorial parameters. The frequencies of methotrexate (p =0.206) and biological agent use (p =0.238) were similar in both JIA groups. Conclusions: To our knowledge, this was the first study to assess lower genital infections in JIA patients allowing the identification of HPV as main cause of cervical dysplasia. Methotrexate and biological agents do not seem to increase risk of lower genital tract infections in JIA patients.

Effects of the use of growth hormone in children and adolescents with juvenile idiopathic arthritis: a systematic review / Efeitos do uso do hormônio de crescimento em crianças e adolescentes com artrite idiopática juvenil: revisão sistemática

Abstract Introduction: Children with juvenile idiopathic arthritis (JIA) often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH) is useful and safe in these patients. Objective: To analyze the effects of GH use in patients with JIA. Method: A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent". Results: Among the 192 identified articles, 20 corresponded to the inclusion criteria. Seventeen longitudinal studies and 3 case reports were found. Most studies analyzed observed increased growth, muscle mass and bone mass using GH. Adverse effects observed were glucose intolerance, diabetes, bone deformities, osteonecrosis, reactivation of the disease and low final height. Conclusion: The majority of studies reported positive effects after the therapeutic use of GH, but some variability in response to treatment was observed. The combination of growth hormone with other drugs seems to be a good option.

Resumo Introdução: Crianças com artrite idiopática juvenil (AIJ) frequentemente apresentam prejuízo no crescimento e baixa estatura. Existem evidências de que o uso terapêutico do hormônio de crescimento (GH) é útil e seguro nesses pacientes. Objetivo: Analisar os efeitos do uso de GH em pacientes com AIJ. Método: Fez-se revisão sistemática da literatura nos últimos 18 anos, nas bases de dados Medline e Embase. Os critérios foram analisados pelos pesquisadores de forma independente. Usaram-se os seguintes descritores: growth hormone, arthritis, juvenile, arthritis, rheumatoid, child e adolescent. Resultados: Entre os 192 artigos identificados, 20 corresponderam aos critérios de inclusão. Foram encontrados 17 estudos longitudinais e três relatos de casos. A maioria dos estudos analisados observou um aumento de crescimento, massa muscular e massa óssea com o uso do GH. Os efeitos adversos observados foram intolerância à glicose, diabetes, deformidades ósseas, osteonecrose, reativação da doença e altura final baixa. Conclusão: A maioria dos estudos relatou efeitos positivos após uso terapêutico do GH, porém certa variabilidade na resposta ao tratamento foi observada. A combinação do hormônio de crescimento com outros medicamentos parece ser uma boa opção.

Coorte Sarar: atividade de doença, capacidade funcional e dano radiológico em pacientes com artrite reumatoide submetidos à artroplastia total de quadril e joelho / Sarar cohort: disease activity, functional capacity, and radiological damage in rheumatoid arthritis patients undergoing total hip and knee arthroplasty

RESUMOObjetivos:A coorte Sarar é composta por pacientes portadores de artrite reumatoide (AR) e artrite idiopática juvenil (AIJ) submetidos a artroplastias de quadril e joelho no hospital Sarah-Brasília. O objetivo deste estudo foi avaliar fatores clínicos e laboratoriais associados à atividade de doença, capacidade funcional e dano radiológico em pacientes com AR, participantes dessa coorte.Métodos:Estudo transversal, com coleta de dados em revisão de prontuário.Resultados:Foram incluídos 32 pacientes, com tempo médio de início da doença de 240 meses. Dezenove pacientes foram submetidos a ATJ e 17, a ATQ. Foi encontrada correlação positiva entre dose máxima de metotrexato (MTX) durante a evolução e Clinical Disease Activity Index (CDAI) (R = -0,46, p = 0,011) e negativa com Simplified Erosion and Narrowing Score (SENS) (R = -0,58, p = 0,004). Valores de SENS foram maiores nos pacientes com fator reumatoide (FR) (p = 0,005) e anticorpo antipeptídeo cíclico citrulinado 3 (anti-CCP3) positivo (p = 0,044), nos com maiores títulos de FR (p = 0,037) e anti-CCP3 (p = 0,025) e menores nos pacientes com história familiar de AR (p = 0,009). Valores de HAQ foram maiores em pacientes mais idosos (p = 0,031). Na regressão linear múltipla, somente “dose máxima de MTX” e “história familiar” permaneceram com associação significativa com SENS (r2= 0,73, p < 0,001 para ambas as variáveis). No modelo que avaliou CDAI, apenas “dose máxima de MTX” permaneceu com associação significativa (r2 = 0,35, p = 0,016).Conclusão:Na coorte Sarar, fatores clínicos e laboratoriais estiveram relacionados à atividade de doença, capacidade funcional e dano radiológico, semelhantemente a estudos que avaliaram pacientes com menor tempo de doença.

ABSTRACTObjectives:The Sarar cohort consists of patients with rheumatoid arthritis and juvenile idiopathic arthritis who underwent hip or knee arthroplasties at hospital SARAH-Brasília. The objective of this study was to evaluate clinical and laboratory factors associated with disease activity, functional capacity and radiological damage in rheumatoid arthritis patients, participants in this cohort.Methods:Cross-secal study, with data collection achieved from medical records review.Results:Thirty-two patients were included, with a mean time of disease onset of 240 months. Nineteen patients underwent total knee and 17 total hip arthroplasty. There was a positive correlation between maximum dose of methotrexate and Clinical Disease Activity Index (R = −0.46, p = 0.011), and a negative one with Simplified Erosion and Narrowing Score (R = −0.58, p= 0.004). Simplified Erosion and Narrowing Score values were higher in patients with rheumatoid factor (p = 0.005) and anti-cyclic citrullinated peptide antibody 3 positivity (p = 0.044), in those with higher rheumatoid factor (p = 0.037) and anti-cyclic citrullinated peptide antibody 3 (p = 0.025) titers, and lower in patients with family history of rheumatoid factor (p = 0.009). Health Assessment Questionnaire values were higher in older patients (p = 0.031). In multiple linear regression, only “maximum dose of methotrexate” and “family history” remained with significant association with Simplified Erosion and Narrowing Score (r2 = 0.73, p < 0.001 for both variables). In the model evaluating “Clinical Disease Activity Index” only “maximum dose of methotrexate” remained significantly associated (r2 = 0.35, p = 0.016).Conclusion:In the Sarar cohort, clinical and laboratory factors were related to disease activity, functional capacity and radiological damage, similar to studies evaluating patients with lower disease duration.

La artritis idiopática juvenil y su relevancia dentro de las enfermedades reumatológicas de la infancia / Juvenile idiopathic arthritis and its relevance in rheumatological diseases of children

El dolor y la inflamación que afecta a las articulaciones o tejidos periarticulares son motivo frecuente de consulta a nivel pediátrico. Dentro de los diagnósticos diferenciales se encuentran las enfermedades reumatológicas. En los últimos años ha cambiado el pronóstico y visión que se tenía de estas enfermedades gracias a los nuevos conocimientos sobre la patogenia y a la incorporación de nuevas terapias con agentes biológicos, basados en anticuerpos monoclonales y que se ha traducido en un cambio en los tratamientos convencionales de estas enfermedades. La Artritis Reumatoidea Juvenil, actualmente denominada Artritis Idiopática Juvenil (AIJ), constituye la enfermedad más frecuente dentro del espectro de estos cuadros autoinmunes. El objetivo de esta presentación es dar a conocer las diversas formas de AIJ y los principales hallazgos clínicos y de laboratorio que pueden orientar al clínico acerca de estas enfermedades y así iniciar un tratamiento oportuno que asegure un buen pronóstico de la enfermedad.

Musculoskeletal pain, joint pain and arthritis are a common complaint in pediatric practice. Among the differential diagnosis for these conditions are rheumatic diseases. Treatment and outcome of these conditions has greatly improved in recent years due to advances in the knowledge of the underlying mechanisms and the development of new therapies with biologic agents, based on monoclonal antibodies. These new therapies have changed the outcome and vision of these diseases. Among the different rheumatologic diseases described in children, Juvenile Rheumatoid Arthritis, now called Juvenile Idiopathic Arthritis (JIA), is the most common disease within the spectrum of autoimmune conditions. The aim of this presentation is to show the different forms of JIA and the main clinical and laboratory findings that can guide the clinician to an early diagnosis and initiate a timely treatment that can guarantee a better prognosis.

Osteoporosis en enfermedades reumáticas / Osteoporosis in rheumatic diseases

La osteoporosis es una enfermedad compleja que resulta de la interacción de células del sistema óseo, principalmente osteoblastos, osteoclastos y osteocitos, que está regulada por varias hormonas y citokinas que interactúan sobre un variado sistema de transducción de señales que derivan en reabsorción o formación de hueso. En varias enfermedades reumáticas este delicado balance se pierde por efecto de la enfermedad misma, aunque también influye poderosamente el tratamiento empleado. En este artículo se revisa el mecanismo general de la fisiología del tejido óseo y su alteración por la inflamación, que son la base de las enfermedades reumáticas. También se revisa la pérdida de masa ósea inducida por esteroides y su tratamiento.

Osteoporosis is a complex disease that results from the interaction of cells in the bone marrow, mainly osteoblasts, osteoclasts and osteocytes, which is regulated by several hormones and cytokines that interact on a variety of signal transduction system that lead to bone resorption or formation. In several rheumatic diseases this delicate balance is lost due to the disease itself, but also strongly influenced by the treatment used. This article reviews the general mechanism of bone physiology and its alteration by inflammation, which are the basis of rheumatic diseases. We also review the bone loss induced by steroid and its treatment.

Inadequação do consumo alimentar de crianças e adolescentes com artrite idiopática juvenil e lúpus eritematoso sistêmico / Inadequate dietary intake of children and adolescents with juvenile idiopathic arthritis and systemic lupus erythematosus

OBJETIVOS: Avaliar o consumo alimentar de crianças e adolescentes com artrite idiopática juvenil (AIJ) e lúpus eritematoso sistêmico (LES) por recordatório de 24 horas e relacioná-lo com características clínicas e antropométricas e com os medicamentos empregados. MÉTODOS: Em estudo transversal, avaliamos os recordatórios de 24 horas de pacientes ambulatoriais. O estado nutricional foi classificado pelo CDC, 2000. Para o cálculo da ingestão, utilizamos o software NutWin UNIFESP-EPM. Para a análise quantitativa e qualitativa, adotamos as Recommended Dietary Allowances e a pirâmide alimentar brasileira. RESULTADOS: A mediana de idade foi 12 na AIJ e 16,5 anos no LES. Na AIJ, 37,5 por cento dos pacientes estavam em atividade de doença, e, no LES, 68,2 por cento tinham Systemic Lupus Erythematosus Disease Activity Index > 4. Foi encontrada desnutrição em 8,3 e 4,5 por cento dos pacientes com AIJ e com LES, respectivamente, e obesidade, em 16,7 e 18,2 por cento. Na AIJ, o consumo excessivo de energia, proteína e lipídios foi de 12,5, 75 e 31,3 por cento, respectivamente. No LES, o consumo excessivo de energia, proteína e lipídios foi de 13,6, 86,4 e 36,4 por cento, respectivamente. Consumo deficiente de ferro, zinco e vitamina A foi observado em 29,2 e 50, 87,5 e 86,4 e 87,5 e 95,2 por cento dos pacientes com AIJ e LES, respectivamente. Não houve relação significante entre consumo, atividade da doença e estado nutricional. CONCLUSÃO: Pacientes com doenças reumáticas apresentam inadequação do consumo alimentar. Ressaltamos a ingestão excessiva de lipídios e proteínas e a ingestão insuficiente de micronutrientes.

OBJECTIVES: To evaluate the dietary intake of children and adolescents with juvenile idiopathic arthritis (JIA) and juvenile systemic lupus erythematosus (JSLE) using a 24-hour diet recall and relating it to the patients clinical and anthropometric characteristics and to the drugs used in their treatment. METHODS: By means of a cross-sectional study, we assessed the 24-hour diet recalls of outpatients. Their nutritional status was classified according to the CDC (2000). The computer program NutWin UNIFESP-EPM was used for food intake calculation. The Recommended Dietary Allowances and the Brazilian food pyramid were used for quantitative and qualitative analysis. RESULTS: Median age was 12 years for JIA patients and 16.5 years for JSLE patients. Among the JIA patients, 37.5 percent had active disease, and among the JSLE patients, 68.2 percent showed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) > 4. Malnutrition was found in 8.3 and 4.5 percent of the JIA and JSLE patients, respectively, and obesity was present in 16.7 and 18.2 percent. For JIA patients, the excessive intake of energy, protein, and lipids was 12.5, 75, and 31.3 percent, respectively. For JSLE patients, the excessive intake of energy, protein, and lipids was 13.6, 86.4, and 36.4 percent, respectively. Low intake of iron, zinc, and vitamin A was found in 29.2 and 50, 87.5 and 86.4, and 87.5 and 95.2 percent of the JIA and JSLE patients, respectively. There was not a significant association between intake, disease activity, and nutritional status. CONCLUSION: Patients with rheumatic diseases have inadequate dietary intake. There is excessive intake of lipids and proteins and low intake of micronutrients.

Clinico-serological profile of juvenile idiopathic arthritis.

We report clinico-serological profile of 210 children with Juvenile idiopathic arthritis (JIA), diagnosed as per ILAR classification criteria. Polyarticular, oligoarticular, and systemic onset disease was observed in 72, 69, and 40 children, respectively. The knee joint was the most frequently involved joint. Antinuclear factor and Rheumatoid factor were positive in 10 and 8, 6 and 20, and 7 and 7 percent children with polyarticular, oligoarticular, and systemic disease, respectively.

Remission status follow-up in children with juvenile idiopathic arthritis

OBJETIVO: Caracterizar a atividade inflamatória articular e sistêmica na artrite idiopática juvenil (AIJ), determinando o estado de remissão com e sem uso de medicação. MÉTODOS: Um total de 165 casos de AIJ, acompanhados em média por 3,6 anos, foram revisados para caracterização de episódios de inatividade, remissão clínica com e sem medicação. Os dados obtidos foram analisados por meio de estatística descritiva, análise de sobrevida, comparação das curvas de Kaplan-Meier, teste de log rank e análise de regressão logística binária para determinação de fatores preditivos para a remissão ou atividade persistente. RESULTADOS: Dos casos revisados, 108 preencheram os critérios de inclusão: 57 pacientes (52,7 por cento) apresentaram um total de 71 episódios de inatividade, com 2,9 anos em média para cada episódio; 36 episódios (50,7 por cento) de inatividade resultaram em remissão clínica sem medicação, sendo 35 por cento do subtipo oligoarticular persistente. A probabilidade de remissão clínica com medicação em 2 anos foi de 81, 82, 97 e 83 por cento para casos de AIJ oligoarticular persistente, oligoarticular estendida, poliarticular e sistêmica, respectivamente. A probabilidade de remissão clínica sem medicação em 5 anos após o início da remissão foi de 40 e 67 por cento para pacientes com AIJ oligoarticular persistente e sistêmica, respectivamente. Houve associação significante da atividade persistente com o uso combinado de medicações para artrite. A idade de início da AIJ foi o único fator preditivo para remissão clínica (p = 0,002). CONCLUSÃO: Nesta coorte, a probabilidade da AIJ evoluir para remissão clínica foi maior nos subtipos oligoarticular persistente e sistêmico, comparados ao curso poliarticular.

OBJECTIVE: To characterize articular and systemic inflammatory activity in juvenile idiopathic arthritis (JIA), identifying remission status with and without medication. METHODS: A total of 165 JIA cases, followed for a mean period of 3.6 years, were reviewed in order to characterize episodes of inactivity and clinical remission on and off medication. The resulting data were analyzed by means of descriptive statistics, survival analysis, by comparison of Kaplan-Meier curves, log rank testing and binary logistic regression analysis in order to identify predictive factors for remission or persistent activity. RESULTS: One hundred and eight of the cases reviewed fulfilled the inclusion criteria: 57 patients (52.7 percent) exhibited a total of 71 episodes of inactivity, with a mean of 2.9 years per episode; 36 inactivity episodes (50.7 percent) resulted in clinical remission off medication, 35 percent of which were of the persistent oligoarticular subtype. The probability of clinical remission on medication over 2 years was 81, 82, 97 and 83 percent for cases of persistent oligoarticular, extended oligoarticular, polyarticular and systemic JIA, respectively. The probability of clinical remission off medication 5 years after onset of remission was 40 and 67 percent for patients with persistent oligoarticular and systemic JIA, respectively. Persistent disease activity was significantly associated with the use of an anti-rheumatic drug combination. Age at JIA onset was the only factor that predicted clinical remission (p = 0.002). CONCLUSIONS: In this cohort, the probability of JIA progressing to clinical remission was greater for the persistent oligoarticular and systemic subtypes, when compared with polyarticular cases.

Assessment of left ventricular systolic and diastolic function in juvenile rheumatoid arthritis.

BACKGROUND AND AIMS: Recognizing the paucity of data regarding echocardiographic studies of Left ventricular (LV) systolic and diastolic function in patients with juvenile rheumatoid arthritis (JRA), a study was carried out to study these parameters in these subjects. SETTINGS, DESIGN AND METHODS: Thirty-five patients with JRA and an equal number of age- and sex-matched controls were studied by two-dimensional and Doppler echocardiography. RESULTS: Patients with JRA had higher systolic and diastolic blood pressures, resting heart rates, LV systolic (26.9+/-4.3 vs. 22.4 +/- 4.1 mm, p=0.001) and diastolic size (42.3+/-4.6 vs. 35.4+/-3.8 mm, p< 0.001) and volumes. Though ejection fraction (EF) and fractional shortening (FS) were normal, they were lower in those with JRA as compared to controls (EF: 62.9+/-4.47 vs. 67.5+/-3.63 %, p< 0.001; FS: 36.4+/-4.5 vs. 38.5 +/- 6.87, p=0.2). On Doppler analysis the JRA group had lower peak E velocity, higher peak A velocity, higher A VTI and more prolonged IVRT. Male patients had higher A VTI and IVRT as compared to females. Those with longer duration of disease had larger LV systolic (r=0.517, p=0.01) and diastolic dimension (r=0.40, p=0.05) and lower FS (r=-0.506, p=0.01). Patients with polyarticular JRA had higher E and A VTI as compared to those with systemic or oligoarticular types. CONCLUSION: Despite an asymptomatic cardiac status, significant systolic and diastolic functional abnormalities exist in patients with JRA. The duration of the disease, mode of presentation, patient's age and gender have a significant impact on the left ventricular systolic and diastolic functions in patients with JRA.

Alteraciones oftalmológicas en pacientes con artritis reumatoide juvenil / Ophthalmological features in subjects with juvenile rheumatoid arthritis

Se realizó un estudio retrospectivo en el Instituto Nacional de Pediatría del año 1993 a 1998 donde se estudiaron 104 expedientes de pacientes con diagnóstico de artritis reumatoide juvenil (ARJ) y se buscaron datos de uveítis, encontrándose 13 pacientes con esta complicación, siendo el sexo femenino con ARJ de tipo pauciarticular y anticuerpos antinucleares positivos el de mayor riesgo; los hombres que presentaron datos de uveítis fueron el grupo que presentó un peor pronóstico visual y mayores complicaciones. La detección temprana y el tratamiento oportuno de todos los pacientes con ARJ sin importar el sexo nos da un mejor pronóstico visual.

Escala EPM-ROM Pediátrica: uma escala reduzida para avaliação da amplitude de movimento articular de pacientes com artrite reumatóide juvenil / Pediatric EPM-ROM scale: a reduced scale to be used in the daily juvenile rheumatoid arthritis practice and in clinical trials

Objetivo: Desenvolver um instrumento específico para a avaliação da amplitude de movimento articular de crianças e adolescentes com artrite reumatóide juvenil (ARJ) e avaliar as suas propriedades de medida. Casuística e métodos: os 10 movimentos da escala Escola Paulista de medicina - Range of Motion (EPM-ROM) Pediátrica originaram-se de 25 movimentos iniciais. A seleção foi baseada em dois critérios: 1 )consenso entre três reumatologistas pediátricos, um fisioterapêuta e um terapeuta ocupacional e 2 )resultados e um estudo piloto envolvendo pacientes com ARJ. A nota obtida em cada movimento articular variou de zero (movimento total) a três (limitação grave) e os ângulos de corte para os movimentos foram obtidos, de modo geral, de acordo com a falta de capacidade em realizar algumas atividades comuns do dia a dia. Foram avaliadas a reprodutibilidade intra-observador (teste-reteste) e a interobservadores. A validade construída foi estudada através da correlação dos valores de alguns parâmetros clínicos rotineiramente utilizados em pacientes com ARJ com as notas da escala EPM-ROM Pediátrica. Resultados: A escala foi aplicada em 34 pacientes com ARJ (11 com tipo de início sistêmico, 11 poliarticular e 12 pauciarticular). A nota obtida (média) foi de 0,57 (0 a 2,05). A reprodutibilidade foi satisfatória. Os coeficientes de correlação de Pearson obtidos entre as notas da escala e os valores dos outros parâmetros (childhood Health Assessment Questionaire, classe funcional do American College of Rheumatology e número de articulações acometidos) variaram entre 0,55 e 0,65 (p<0,05). Conclusão: A escala EPM-ROM Pediátrica é um instrumento válido e reprodutível e é útil na avaliação de pacientes com ARJ

The effect of methotrexate and azathioprine on the serum levels of IgA-alfa1-antitrypsin complex in juvenile chronic arthritis

In the present study we investigated the influence of methotrexate (MTX) and azathioprine (AZA) on the serum levels of the IgA-alpha1-antitypsin (IgA-AT) complex in patients with the systemic form of juvenile chronic arthritis (JCA). Fifty-six JCA patients (22 treated with MTX, 18 treated with AZA, and 16 not treated with any immuno-suppressive agent) were enrolled in the study. MTX dosage ranged from 0.3 to 0.5 mg kg(-1) week(-1) while AZA was given daily at an average dose of 1 mg/kg. MTX was given for 13 months (SD = 7 months) whereas AZA for 11 months (SD = 6 months). The average value of the complex was higher in JCA patients than in both control groups (0.74 + 0.73 U vs 0.37 + 0.13 U (control children), P<0.001 and vs 0.23 + 0.12 U (control adults), P<0.001). Values exceeding the normal range were found in twenty-two JCA patients (39.4 percent). Serum IgA-AT level was lowest in the MTX group compared to AZA and non-treated patients (0.56 + 0.24 U, 0.76 + 0.43 U, 0.95 + 0.52 U, respectively, P<0.05). IgA values exceeding normal levels for age were found in 14 percent of the patients. A correlation between the levels of the IgA-AT complex and C-reactive protein (r = 0.43, P<0.01), alpha1-acid-glycoprotein (r = 0.45, P<0.01), alpha1-antichymotrypsin (r = 0.52, P<0.01), alpha1-antitrypsin (r = 0.40, P<0.01) and IgA (r = 0.56, P<0.01) was established.