Tuberculosis miliar en pacientes en tratamiento con adalimumab, una asociación no tan frecuente en pediatría / Miliary tuberculosis in patients treated with adalimumab, an association not so frequent in pediatrics

Los antagonistas del Factor de Necrosis Tumoral-α, son medicamentos que en los últimos años han tenido un incremento de su uso en pacientes con condiciones inflamatorias inmunomediadas en pediatría, como la Artritis Idiopática Juvenil y la Enfermedad Inflamatoria Intestinal. El uso de estos medicamentos en adultos tiene una fuerte asociación con la primoinfección o reactivación por Mycobacterium tuberculosis, pero en niños la evidencia es limitada. Se presentan 2 casos de pacientes tratados con adalimumab, quienes, a pesar de un buen control de su enfermedad y una prueba de tuberculina negativa al inicio de la terapia, desarrollaron tuberculosis miliar en el seguimiento, con importantes implicaciones para su salud. El tamizaje de tuberculosis latente con tuberculina/IGRAS (Interferón-γ release assays, por sus siglas en inglés) y un alto índice de sospecha de tuberculosis, son las herramientas disponibles para una adecuada identificación de la tuberculosis en pacientes que reciben crónicamente estas terapias.

Tumor Necrosis Factor-α antagonists are drugs that in recent years have seen an increase in their use in patients with immune-mediated inflammatory conditions in pediatrics such as Juvenile Idiopathic Arthritis and Inflammatory Bowel Disease. The use of these drugs in adults has a strong association with primary infection or reactivation by mycobacterium tuberculosis, but in children the evidence is limited. We present 2 cases of patients treated with adalimumab who, despite good control of their disease and a negative tuberculin test at the beginning of therapy, developed miliary tuberculosis during follow-up with important implications for their health. Screening for latent tuberculosis with tuberculin / IGRAS (Interferón-γ release assays) and a high index of suspicion for tuberculosis are the tools available for an adequate identification of tuberculosis in patients who receive these therapies chronically.

Quality of life in children and adults with Idiopathic juvenile arthritis: cross-sectional study in Brazilian patients / Qualidade de vida em crianças e adultos com artrite idiopática juvenil: estudo transversal em pacientes brasileiros

ABSTRACT BACKGROUND AND OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease which may persist into adulthood. Pain and joint deformities affect quality of life (QoL). The objective was to study the influence of JIA in QoL of children and adults, comparing the two groups. METHODS: Cross-sectional study of 47 JIA patients (20 children and 27 adults) using the following questionnaires: Juvenile Arthritis Multidimensional Assessment Report (JAMAR), Health Assessment Questionnaire (HAQ) in adults and Childhood Health Assessment Questionnaire CHAQ) in children. Epidemiological, clinical and treatment data was collected. RESULTS: No differences were found in gender, arthritis subset, presence of uveitis and fever, visual analog scale for pain and duration of morning stiffness. Adult patients required more biologics in the treatment (p=0.02) and had higher age at disease onset than children (p=0.001). About 45% of children and 51.8% of adults complained having pain and 40% of children and 48% of adults were not satisfied with the current status of their disease. CONCLUSION: Children and adult patients with JIA had similar degree of pain, morning stiffness and functional impairment. Functional impairment was considered mild but the level of pain found was higher than in other studies. Almost half of patients were not satisfied with the treatment.

RESUMO JUSTIFICATIVA E OBJETIVOS: A artrite idiopática juvenil (AIJ) é uma doença reumática crônica que pode persistir na idade adulta. Dor e deformidades articulares afetam a qualidade de vida (QV). O objetivo foi estudar a influência da AIJ na QV de crianças e adultos, comparando os dois grupos. MÉTODOS: Estudo transversal de 47 pacientes com AIJ (20 crianças e 27 adultos) utilizando os questionários: Juvenile Arthritis Multidimensional Assessment Report (JAMAR), Health Assessment Questionnaire (HAG) em adultos e Childhood Health Assessment Questionnaire (CHAQ) em crianças. Dados epidemiológicos, clínicos e de tratamento foram coletados. RESULTADOS: Não foram encontradas diferenças em sexo, subconjunto de artrite, presença de uveíte e febre, escala analógica visual para dor e duração da rigidez matinal. Pacientes adultos necessitaram mais fármacos biológicos no tratamento (p=0,02) e tinham maior idade de início da doença do que crianças (p=0,001). Cerca de 45% das crianças e 51,8% dos adultos queixaram-se de dor e 40% das crianças e 48% dos adultos não estavam satisfeitos com o estado atual de sua doença. CONCLUSÃO: Pacientes crianças e adultos com AIJ apresentaram graus semelhantes de dor, rigidez matinal e comprometimento funcional. O comprometimento funcional foi considerado leve, mas o nível de dor encontrado foi maior do que em outros estudos. Quase metade dos pacientes não ficou satisfeita com o tratamento.

Artritis Idiopática Juvenil: manifestaciones clínicas y tratamiento / Juvenile Idiopathic Arthritis: clinical manifestation and treatment

Resumen La Artritis Idiopática Juvenil es la enfermedad reumática más frecuente en niños. Es una enfermedad crónica, degenerativa y de etiología desconocida; que puede dejar múltiples secuelas en la población pediátrica. Consta de siete afecciones definidas por la International League of Associations for Rheumatology del 2001: Artritis Sistémica, Oligoartritis, Artritis con Factor Reumatoide positivo o Factor Reumatoide negativo, Artritis relacionada a entesitis, Artritis psoriasica y Artritis indiferenciada; distintas tanto en el aspecto clínico, patogénico como evolutivo. Esta enfermedad se caracteriza por una alteración de la regulación del sistema inmunitario innato con una falta de linfocitos T autorreactivos y autoanticuerpos. La inflamación continua estimula el cierre rápido y prematuro del cartílago de crecimiento provocando un acortamiento óseo. Para llegar a su diagnóstico no se requiere más que una buena historia clínica y examen físico, ya que no hay laboratorios o gabinete lo bastante sensible que nos puedan ayudar. Fármacos como el metrotexate y los inhibidores del factor de necrosis tumoral han venido a modificar la evolución de la enfermedad y mejorar la calidad de vida de estos pacientes.

Abstract Juvenile idiopathic arthritis is the most common rheumatic disease in children. It is a chronic and degenerative disease, with an unknown etiology; that can leave multiple sequels in the pediatric population. There are seven conditions defined by 2001 International League of Associations for Rheumatology: Systemic Arthritis, Oligoarthritis, Arthritis with positive rheumatoid factor or negative rheumatoid factor, enthesitis-related arthritis and undifferentiated arthritis; distinct in clinical, pathogenetic and evolutionary aspects. This disease is characterized by an alteration on the regulation of the innate immune system with a lack of autoreactive lymphocytes T and autoantibodies. Continuous inflammation stimulates the rapid and premature closure of the growth cartilage causing bone shortening. To arrive at the diagnosis, it is only necessary to have a good medical history and physical exam, since there are no laboratory test sensitive enough to help us. Drugs such as methotrexate and tumor necrosis factor inhibitors have come to modify the evolution of the disease and improve the quality of life of these patients.

Cardiac autonomic modulation in juvenile idiopathic arthritis with use of biological medication: relato de caso / Modulação autonomica cardíaca na artrite idiopática juvenil com uso de medicação biológica

INTRODUCTION: The introduction of biological medication in Juvenile Idiopathic Arthritis (JIA) proposes better therapeutic results with decreased pain and inflammation and consequent reduction in joint damage. The autonomic state can be a predictor for verifying the response to immunomodulation therapy. Thus, measuring heart rate variability can express autonomous behavior and possibly accompany the response to therapy through the expression of the inflammatory conditionObjectiveAnalysis of heart rate variability in a child with Juvenile Idiopathic Arthritis using the anti-Tumor Necrosis Factor.METHODS: This is a clinical case report of an 8-year-old male with a diagnosis of JIA - oligoarticular form, using etanercept, admitted to Clínica Escola de Fisioterapia UNINORTE, Acre, Brazil in 2017. We analyzed laboratory and imaging tests, kinetic-functional evaluation, examination of cardiac autonomic modulation and physiotherapeutic treatment for analgesic, anti-inflammatory purposes, gaining flexibility, strength and postural re-education, according to CARE guidelines, case report guidelinesRESULTS: After medication administration, there was a decrease in pain and normalization of serum creatinine (0.50 mg / dL) and CRP (less than 6 mg / dL) and an increase in erythrocyte sedimentation rate (17 mm3). In the examination of heart rate variability, the linear indices in the time domain showed a predominance of parasympathetic activity (RMSSD: 35ms), with decreased sympathetic control measured through the frequency domain (LF: 27.1 un). However, non-linear methods showed low variability with little dispersion of RR intervalsCONCLUSION: In the present report, the linear indices showed parasympathetic predominance and in the non-linear analysis a low heart rate variability with abnormal and insufficient adaptation of the autonomic nervous system in a child with juvenile idiopathic arthritis using biological medication

INTRODUÇÃO: A introdução de medicamentos biológicos na Artrite Idiopática Juvenil (AIJ) propõe melhores resultados terapêuticos com diminuição da dor e inflamação e consequente redução no dano articular. O estado autonômico pode ser um preditor para verificar a resposta à terapia de imunomodulação. Assim, medir a variabilidade da frequência cardíaca pode expressar um comportamento autônomo e possivelmente acompanhar a resposta à terapia através da expressão da condição inflamatóriaOBJETIVO: Análise da variabilidade da frequência cardíaca em uma criança com artrite idiopática juvenil utilizando o Fator de Necrose TumoralMÉTODO: Este é um relato de caso clínico de um homem de 8 anos com diagnóstico de AIJ - forma oligoarticular, usando etanercept, admitido na Clínica Escola de Fisioterapia UNINORTE, Acre, Brasil em 2017. Analisamos testes laboratoriais e de imagem, cinéticos - avaliação funcional, exame da modulação autonômica cardíaca e tratamento fisioterapêutico para fins analgésicos e anti-inflamatórios, ganhando flexibilidade, força e reeducação postural, de acordo com as diretrizes da CARE, diretrizes de relato de casoRESULTADOS: Após a administração da medicação, houve diminuição da dor e normalização da creatinina sérica (0,50 mg/dL) e PCR (menos de 6 mg/dL) e aumento da taxa de sedimentação de eritrócitos (17 mm3). No exame da variabilidade da frequência cardíaca, os índices lineares no domínio do tempo mostraram predominância da atividade parassimpática (RMSSD: 35ms), com diminuição do controle simpático medido pelo domínio da frequência (LF: 27,1 un). Entretanto, métodos não lineares apresentaram baixa variabilidade com pouca dispersão dos intervalos RRCONCLUSÃO: No presente relatório, os índices lineares mostraram predominância parassimpática e, na análise não linear, baixa variabilidade da frequência cardíaca com adaptação anormal e insuficiente do sistema nervoso autônomo em criança com artrite idiopática juvenil em uso de medicação biológica

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)

Effects of hydrokinesiotherapy in pain, trophism and muscle strength in a child with juvenile idiopathic arthritis. Case report / Efeitos da hidrocinesioterapia na dor, no trofismo e na força muscular de uma criança com artrite idiopática juvenil. Relato de caso

ABSTRACT BACKGROUND AND OBJECTIVES: Juvenile idiopathic arthritis is a childhood rheumatic disease, which can interfere with the trophism and muscular strength of the individual due to persistent pain. Hydrokinesiotherapy may be an alternative in the management of this disease. The objective of this study was to verify the effects of hydrokinesiotherapy on pain, trophism and muscular strength of a child with juvenile rheumatoid arthritis. CASE REPORT: Female patient, 12 years old, diagnosed with juvenile rheumatoid arthritis one year ago. The pain was evaluated by the visual analog scale and the body pain map, the muscular trophism by the perimetry of the arms and thighs, and the muscular strength by isokinetic dynamometry at a speed of 240º. The hydrokinetic therapeutic intervention program (adaptation, warm up, mobility and flexibility, muscle strengthening, cardiorespiratory fitness, balance and proprioception, and relaxation) was carried from October to December 2017, once a week, for 1 hour, totaling 10 sessions. At the end, there was a decrease in pain by 2.7 points (moderate to mild), an increase in muscle trophism of the arms and right thigh in 1cm and an increase in the torque peak (progress ranging from 12.3 to 37.9%) and total work (progress ranging from 18.6 to 76.7%) in all muscle groups analyzed in both knees. CONCLUSION: The hydrokinetic therapeutic intervention plan shown to be an effective strategy to alleviate the pain and increase trophism and muscle strength of the individual with juvenile idiopathic arthritis.

RESUMO JUSTIFICATIVA E OBJETIVOS: A artrite idiopática juvenil é uma doença reumática da infância, que pode interferir no trofismo e na força muscular do indivíduo, devido à dor persistente. A hidrocinesioterapia pode ser uma alternativa no manuseio dessa doença. O objetivo deste estudo foi verificar os efeitos da hidrocinesioterapia na dor, no trofismo e na força muscular de uma criança com artrite reumatoide juvenil. RELATO DO CASO: Paciente do sexo feminino, 12 anos de idade, diagnosticada com artrite reumatoide juvenil há um ano. Avaliou-se a dor por meio da escala analógica visual e do mapa de dor corporal; o trofismo muscular, por meio da perimetria dos braços e das coxas; e a força muscular, por meio da dinamometria isocinética em velocidade de 240º. O programa de intervenção hidrocinesioterapêutica (adaptação, aquecimento, mobilidade e flexibilidade, fortalecimento muscular, condicionamento cardiorrespiratório, equilíbrio e propriocepção e relaxamento) foi realizado nos meses de outubro a dezembro de 2017, uma vez por semana, durante 1 hora, totalizando 10 sessões. Ao fim, houve diminuição da dor em 2,7 pontos (de moderada a leve), aumento do trofismo muscular dos braços e da coxa direita em 01 cm e aumento do pico de torque (progresso que variou entre 12,3 e 37,9%) e do trabalho total (progresso que variou entre 18,6 e 76,7%) em todos os grupos musculares analisados, de ambos os joelhos. CONCLUSÃO: O plano de intervenção hidrocinesioterapêutica mostrou-se como uma estratégia eficaz para o alívio da dor e aumento do trofismo e da força muscular do indivíduo com artrite idiopática juvenil.

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.

Diagnóstico de artritis idiopática juvenil en el primer nivel de atención: a propósito de un caso / Diagnosis of juvenile idiopathic arthritis in the first level of care: in relation to a case

Introducción: la artritis idiopática juvenil es una afección inflamatoria y sistémica que afecta a pacientes menores de 18 años. Engloba una serie de manifestaciones clínicas que en edad adulta tiene nombre común como enfermedades ya establecida; sin embargo, todas ellas se engloban bajo este término en niños y adolescentes. Todas sus formas clínicas tienen como elemento común la presencia de un cuadro inflamatorio que genera artritis; según la característica de la toma articular y la presencia de otras manifestaciones, es que se definen las formas clínicas de la enfermedad. Objetivo: dar a conocer los elementos clínicos y de laboratorio que permiten llegar al diagnóstico de la artritis idiopática juvenil en la atención primaria de salud. Caso clínico: se presenta el caso de una paciente de 9 años de edad que presenta manifestaciones clínicas y de laboratorio que permiten realizar el diagnóstico de artritis idiopática juvenil. Conclusiones: las enfermedades reumáticas afectan generalmente a pacientes adultos, con predominio de edades avanzadas, sin embargo, resulta importante conocer los elementos diagnósticos de cada uno de ellos para poder detectar su aparición en edades tempranas de la vida(AU)

Introduction: juvenile idiopathic arthritis is an inflammatory and systemic condition that affects patients under 18 years of age. It encompasses a series of clinical manifestations that in adult age has a common name as established diseases; however, all of them are included under this term in children and adolescents. All its clinical forms have as a common element the presence of an inflammatory condition that generates arthritis; According to the characteristic of the joint taking and the presence of other manifestations, it is that the clinical forms of the disease are defined. Objective: to present the clinical and laboratory elements that allow to reach the diagnosis of juvenile idiopathic arthritis in primary health care. Clinical case: the case of a 9-year-old patient with clinical and laboratory manifestations that allow the diagnosis of juvenile idiopathic arthritis is presented. Conclusions: rheumatic diseases generally affect adult patients, with a predominance of advanced ages; however, it is important to know the diagnostic elements of each of them in order to detect their appearance at early ages of life(AU)

An Interesting Case Of Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis can be difficult to diagnose because there are no specific diagnostic tests and arthritis is often not evident early in the course. We present a case of systemic juvenile idiopathic arthritis involving multiple small and large joints along with flexion contractures. Investigations revealed iron deficiency anemia and a positive rheumatoid factor. Patient was started on DMARDs, corticosteroids and iron supplements.

Enthesitis-related Arthritis

Enthesitis-related arthritis (ERA) is a disease predominantly affecting the joints and entheses of the lower extremities and has the potential to eventually affect the sacroiliac joints and spine evolving to juvenile ankylosing spondylitis. ERA is also characterized by rheumatoid factor seronegativity, paucity of antinuclear antibody, and a strong association with the human leukocyte antigen-B27. ERA accounts for a higher proportion of juvenile idiopathic arthritis (JIA) cases in the Asian population compared to other populations. Advances in the understanding of ERA pathogenesis continue to progress and have led to the development of new treatments targeting pro-inflammatory cytokines. In particular, tumor necrosis factor-α inhibitors have become a mainstay of therapy for patients in whom therapy with anti-inflammatory drugs and/or disease-modifying anti-rheumatic drugs are inadequate or contraindicated. Compared to other JIA subtypes, ERA is associated with a poorer quality of life, worse function, and a higher likelihood of ongoing active disease after the initial treatment. Because the current guidelines for the management of ERA is not considered separately from other categories of JIA, there is a need for treatment guidelines specific to ERA to improve the overall disease outcomes.

Inmunizaciones en pacientes con artritis idiopática juvenil / Immunizations in patients with juvenile idiopathic arthritis

Los pacientes con AIJ/ARJ presentan un riesgo mayor de infecciones inmunopreveni-bles, debido a su disfunción inmune, exacerbada por la actividad de su enfermedad y la terapia inmunosupresora. Las vacunas inactivadas han demostrado un perfil de seguridad adecuado en estos pacientes, por lo que no están contraindicadas, aunque su respuesta inmune puede ser inadecuada. Las vacunas vivas atenuadas, formal-mente contraindicadas, poseen una información creciente que permite evaluar su riesgo beneficio de manera individual. Por este motivo, debemos procurar mantener el calendario de vacunas actualizado y complementado, evitando el retraso en esque-mas de vacunación y poniéndolo al día lo antes posible, con estrategias basadas en el individuo, idealmente antes de iniciar la terapia inmunosupresora o de lo contrario durante ella. Para llevar a cabo esto debemos conocer y considerar los intervalos entre las vacunas, los esquemas acelerados, la solicitud de vacunas especiales, las aprobaciones vigentes y, finalmente, sus contraindicaciones.

Patients with JIA/JRA present a higher risk of vaccine-preventable infections, due to their immune dysfunction, exacerbated by the activity of their disease and immu-nosuppressive therapy. Inactivated vaccines have shown an adequate safety profile in these patients, so they are not contraindicated, although their immune response may be impaired. Live attenuated vaccines, formally contraindicated, have a growing information that allows to evaluate their risk benefit case by case. For this reason we must try to keep the vaccination schedule updated and supplemented, avoiding the delay in vaccination schemes and updating it as soon as possible, with taylor-based strategies, ideally before starting immunosuppressive therapy or otherwise during it. To carry out this we must manage and consider the intervals between the vaccines, the accelerated schemes, the request for special vaccines, the current approvals and, finally, their contraindications.

Values of fat saturation sequence in MRI for juvenile arthritis / 中南大学学报(医学版)

Objective:To explore the values of fat saturation sequence in MRI for juvenile arthritis.Methods:A total of 1 131 cases with juvenile arthritis and 1 601 with symptomatic arthritis were examined by MRI normal T1 weighted imaging (T1WI) and T2 weighted imaging (T2WI)sequence and spectral presaturation attenuatedinversion recovery (SPAIR) T2 fat saturation sequence.All the images were independently evaluated by two senior doctors from the Department of Radiology and the Department of Pediatric Rheumatology and Immunology respectively to confirm the types and degree of pathological changes of joint tissues.Results:Among the subjects,847 patients demonstrated positive in MRI,accounting for 52.9％;409 patients showed positive in normal sequence,accounting for 48.3％;816 patients showed positive in fat saturation sequence,accounting for 96.3％.Joint hydrops accounted for 59.5％.Bone marrow edema accounted for 39.7％.The relevant ratio of bone marrow edema,joint hydrops,thickening of synovium and cartilage injuries in fat saturation sequence were higher than that in normal sequence (P＜0.05).The relevant ratio of bone erosion in normal sequence was higher than that in fat saturation sequence (P＜0.05).However,no significant difference of joint cysts was found between the fat saturation sequence and normal sequence (P＜0.05).Conclusion:Application of fat saturation sequence by MRI to check juvenile arthritis could obviously improve the positive MRI relevant ratio.In addition,the relevant ratio of the early pathological changes of juvenile arthritis (such as bone marrow edema and joint hydrops) was high,which might provide references for the early diagnosis of juvenile arthritis.

Human Leukocyte Antigen B27 and Juvenile Idiopathic Arthritis and Classification of Juvenile Spondyloarthropathies by the Assessment of SpondyloArthritis International Society Criteria

OBJECTIVE: We examined the clinical relationship between human leukocyte antigen B27 (HLA-B27) and juvenile idiopathic arthritis (JIA). Additionally, we assessed the usefulness of the Assessment of SpondyloArthritis International Society (ASAS) criteria for diagnosing juvenile spondyloarthropathies (SpA). METHODS: We retrospectively reviewed medical records of 239 patients with JIA classified according to the International League of Associations for Rheumatology (ILAR) classification to analyze the features of the joint involvement site. Results were correlated with the presence of HLA-B27. After that, we classified the 239 JIA patients according to the ASAS criteria to diagnose juvenile SpA. The relationship between the ASAS criteria and a diagnosis of juvenile SpA was analyzed by a chi-squared test. RESULTS: Back pain was associated with HLA-B27 in boys (p=0.002) but not in girls (p=0.616). In both sexes, involvement of the small joints in the lower extremities was highly associated with HLA-B27 (p=0.001 for boys, p=0.021 for girls). In addition, HLA-B27 was associated with enthesitis (p=0.004 for boys, p=0.021 for girls). Eighty-seven (36.4%) patients with JIA fulfilled the ASAS criteria; 2 (0.8%) had axial SpA and 85 (35.6%) had peripheral SpA. HLA-B27 was the most significant factor for diagnosing juvenile SpA (sensitivity 80%, specificity 99.31%, positive likelihood ratio, 116). CONCLUSION: The ILAR criteria have some weaknesses for diagnosing HLA-B27-positive JIA patients in early stages. The use of the ASAS criteria for juvenile patients will enable pediatric rheumatologists to diagnose juvenile SpA patients earlier.

Comparação entre o Disease Activity Score-28 e o Juvenile Arthritis Disease Activity Score na artrite idiopática juvenil / Comparison of the Disease Activity Score and Juvenile Arthritis Disease Activity Score in the juvenile idiopathic arthritis

Introdução A avaliação de atividade da artrite reumatoide e da artrite idiopática juvenil é feita por meio de instrumentos distintos, respectivamente pelo DAS-28 e pelo JADAS. Objetivo Comparar o DAS-28 e o JADAS com a pontuação de 71, 27 e 10 articulações, na artrite idiopática juvenil. Método Foram avaliadas 178 visitas em oito pacientes com artrite idiopática juvenil, participantes de um ensaio clínico controlado de fase III, testando eficácia e segurança do abatacepte. Pontuaram-se as articulações ativas e limitadas, a avaliação global pelo médico e pelos pais em escala analógica visual de 0-10 cm e a velocidade de hemossedimentação convertida em escala de 0-10, em todas as visitas. A comparação entre os índices de atividade entre diferentes observações foi por Anova ou modelo ajustado Gama. As observações pareadas entre o DAS-28 e o JADAS 71, 27 e 10, respectivamente, foram analisadas por meio de regressão linear. Resultados Houve diferença significativa entre as medidas individuais, exceto a VHS, nos primeiros quatro meses de tratamento com biológico, quando cinco entre os oito pacientes atingiram a resposta ACR-Pedi 30, com melhora. Os índices DAS-28, JADAS 71, 27 e 10 também apresentaram diferença relevante durante o período de observação. O ajustamento por meio de regressão linear entre o DAS-28 e o JADAS resultou em fórmulas matemáticas para conversão: [DAS-28 = 0,0709 (JADAS 71) + 1,267] (R2 = 0,49); [DAS-28 = 0,084 (JADAS 27) + 1,7404] (R2 = 0,47) e [DAS-28 = 0,1129 (JADAS-10) + 1,5748] (R2 = 0,50). Conclusão A conversão da pontuação do DAS-28 e do JADAS 71, 27 e 10 por esse modelo matemático permitiria a aplicação equivalente de ambos em adolescentes com artrite. .

Introduction The assessment of the activity of rheumatoid arthritis and juvenile idiopathic arthritis is made by means of the tools DAS-28 and JADAS, respectively. Objective To compare DAS-28 and JADAS with scores of 71, 27 and 10 joint counts in juvenile idiopathic arthritis. Method A secondary analysis of a phase III placebo-controlled trial, testing safety and efficacy of abatacept was conducted in 8 patients with 178 assessment visits. Joint count scores for active and limited joints, physician's and parents’ global assessment by 0–10 cm Visual Analog Scale, and erythrocyte sedimentation rate normalized to 0–10 scale, in all visits. The comparison among the activity indices in different observations was made through Anova or adjusted gamma model. The paired observations between DAS-28 and JADAS 71, 27 and 10, respectively, were analyzed by linear regression. Results There were significant differences among individual measures, except for ESR, in the first 4 months of biological treatment, when five of the eight patients reached ACR-Pedi 30, with improvement. The indices of DAS-28, JADAS 71, 27 and 10 also showed significant difference during follow-up. Linear regression adjusted model between DAS-28 and JADAS resulted in mathematical formulas for conversion: [DAS-28 = 0.0709 (JADAS 71) + 1.267] (R2 = 0.49); [DAS-28 = 0.084 (JADAS 27) + 1.7404] (R2 = 0.47) and [DAS-28 = 0.1129 (JADAS-10) + 1.5748] (R2 = 0.50). Conclusion The conversion of scores of DAS-28 and JADAS 71, 27 and 10 for this mathematical model would allow equivalent application of both in adolescents with arthritis. .

Epidemiology of juvenile arthritis in the world / 中华实用儿科临床杂志

Objective To review epidemiological studies of juvenile arthritis (JA) published in nearly 20 years,and improve the understanding of JA among medical workers.Methods A review of 31 epidemiological studies of JA from 1992 to 2012,among 23 countries and 37 regions were undertaken.Results Annual incidence rate(AIR) (including JIA/JRA/JCA) was reported as 0.83 per 100 000 children to 25.0 per 100 000 children,the highest AIR was Scotland(25.0/100 000),the lowest was Japan (0.83/100 000).Prevalence of JA was reported as 3.8 per 100 000 children to 367.0 per 100 000 children.The highest one was Australia(367.0/100 000),the lowest was Taiwan(3.8/100 000).The prevalence and incidence had a wide fluctuation range.The major factors contributing to differences in estimates included:(1) Different diagnostic criterias among different countries and regions ; (2) Physicians of different levels and experiences had vary awareness of JA,lead to vary errors about the clinical diagnosis ; (3) Vary in economic levels,knowledge,family factors and health care resources led to different attention to JA,and had attendance rates of JA ; (4) Different national,regional,ethnic,and other causes lead to the inherent difference about epidemiological characteristics of JA ; (5) It may exist statistical errors.Conclusions Many factors contribute to the diversity prevalence and incidence of JA among different countries and regions.The actual result need unified diagnosis and classification criteria,rheumatoid specialist physicians,larger samples,multi-center epidemiological survey research data.

Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis / 소아과

Juvenile rheumatoid arthritis (JRA) is the most common rheumatic childhood disease; its onset is before 16 years of age and it persists for at least 6 weeks. JRA encompasses a heterogeneous group of diseases that is classified according to 3 major presentations: oligoarthritis, polyarthritis, and systemic onset diseases. These presentations may originate from the same or different causes that involve interaction with specific immunogenetic predispositions, and result in heterogeneous clinical manifestations. An arthritic joint exhibits cardinal signs of joint inflammation, such as swelling, pain, heat, and loss of function; any joint can be arthritic, but large joints are more frequently affected. Extra-articular manifestations include high fever, skin rash, serositis, and uveitis. The first 2 types of JRA are regarded as T helper 1 (Th1) cell-mediated inflammatory disorders, mainly based on the abundance of activated Th1 cells in the inflamed synovium and the pathogenetic role of proinflammatory cytokines that are mainly produced by Th1 cell-stimulated monocytes. In contrast, the pathogenesis of systemic onset disease differs from that of other types of JRA in several respects, including the lack of association with human leukocyte antigen type and the absence of autoantibodies or autoreactive T cells. Although the precise mechanism that leads to JRA remains unclear, proinflammatory cytokines are thought to be responsible for at least part of the clinical symptoms in all JRA types. The effectiveness of biologic therapy in blocking the action of these cytokines in JRA patients provides strong evidence that they play a fundamental role in JRA inflammation.

Treatment of juvenile rheumatoid arthritis / 소아과

The systematic approach to pharmacologic treatment is typically to begin with the safest, simplest, and most conservative measures. It has been realized that the more rapidly inflammation is under control, the less likely it is that there will be permanent sequelae. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the mainstay of initial treatment for inflammation. In addition, the slow-acting antirheumatic drugs (SAARDs) and disease-modifying antirheumatic drugs (DMARDs) have efficacy of anti-inflammatory action in children with chronic arthritis. New therapeutic modalities for inflammation, such as etanercept and infliximab, promise even further improvements in the risk/benefit ratio of treatment. It is not typically possible at the onset of the disease to predict which children will recover and which will go on to have unremitting disease with lingering disability or enter adulthood with serious functional impairment. Therefore, the initial therapeutic approach must be vigorous in all children.

Immunization consensus for children and adolescents with rheumatic diseases / Consenso de imunização para crianças e adolescentes com doenças reumatológicas

Crianças e adolescentes com doenças reumatológicas apresentam maior prevalência de doenças infecciosas quando comparados com a população em geral, em decorrência de atividade da doença, possível deficiência imunológica secundária à própria doença, ou uso de terapia imunossupressora. A vacinação é uma medida eficaz para a redução da morbidade e mortalidade nesses pacientes. O objetivo deste artigo foi realizar um consenso de eficácia e segurança das vacinas em crianças e adolescentes com doenças reumatológicas infantis baseadas em níveis de evidência científica. Imunização passiva para os pacientes e orientações para as pessoas que convivem com doentes imunodeprimidos também foram incluídas. Os 32 pediatras reumatologistas membros do Departamento de Reumatologia da Sociedade de Pediatria de São Paulo (SPSP) e/ou da Comissão de Reumatologia Pediátrica da Sociedade Brasileira de Reumatologia elaboraram o consenso, sendo que alguns desses profissionais estão envolvidos em pesquisas e publicações científicas nesta área. A pesquisa dos termos eficácia e/ou segurança das diferentes vacinas em crianças e adolescentes com doenças reumatológicas foi realizada nas bases de Medline e Scielo, de 1966 até março de 2009, incluindo revisões, estudos controlados e relatos de casos. O grau de recomendação e o nível científico de evidências dos estudos foram classificados em quatro níveis para cada vacina. De um modo geral, as vacinas inativadas e de componentes são seguras nos pacientes com doenças reumatológicas, mesmo em uso de terapias imunossupressoras. Entretanto, vacinas com agentes vivos atenuados são, em geral, contraindicadas para os pacientes imunossuprimidos.

Incidence of infectious diseases is higher in children and adolescents with rheumatic diseases than in the general population due to disease activity, possible immune deficiency secondary to the disease itself, or the use of immunosuppressive drugs. Vaccination is effective in reducing morbidity and mortality in those patients. The objective of this study was to establish an evidence-based consensus on the efficacy and safety of vaccination in children and adolescents with rheumatic diseases. Passive immunization of patients and guidelines for people who live with immunosuppressed patients were also included. The 32 pediatric rheumatologists of the Rheumatology Department of the Pediatrics Society of São Paulo, (SPSP, from the Portuguese), São Paulo, SP, Brazil, and/or the Commission on Pediatrics Rheumatology of the Brazilian Society of Rheumatology are responsible for this consensus; some of those professionals are involved on research and scientific publications in this field. The words efficacy and/or safety of different vaccines in children and adolescents with rheumatologic diseases were searched in Medline and Scielo data bases from 1966 to March 2009, including reviews, controlled studies, and case reports. The degree of recommendation and the scientific evidence of the studies were classified in four levels for each vaccine. As a rule, inactive and protein components vaccines are safe for patients with rheumatologic diseases, even in the presence of immunosuppressive therapy. However, live attenuated vaccines are, in general, contraindicated for immunosuppressed patients.

Association of alkaline phosphatase phenotypes with arthritides.

Arthritides, a symmetrical polyarticular disease of the bone are a heterogenous group of disorders in which hereditary and environmental factors in combination with an altered immune response appear to play a causative and pathogenic role in its occurrence. Alkaline phosphatase (ALP) is an enzyme found in all tissues, with particularly high concentrations of ALP observed in the liver, bile ducts, placenta, and bone.Alkaline phosphatase is an orthophosphoric monoester phosphohydrolase catalyzing the hydrolysis of organic esters at alkaline pH, indicating that alkaline phosphatase is involved in fundamental biological processes.1 The present study envisages on identifying the specific electromorphic association of alkaline phosphatase with arthritides. Phenotyping of serum samples was carried out by PAGE (Polyacrylamide gel electrophoresis) following Davies (1964)2 protocol on 41 juvenile arthritis, 150 rheumatoid arthritis and 100 osteo arthritis apart from, 25 normal children and 100 adult healthy subjects. Phenotyping of alkaline phosphatase revealed an increase in preponderance of p+ and p++ phenotypes in juvenile, rheumatoid and osteo arthritic patients. However a significant association of these phenotypes was observed only with rheumatoid arthritis condition (c2:17.46). Similarly, a significant increase of p+ phenotypes in female rheumatoid arthritis patients was observed (c2:14.973), suggesting that the decrease in p° (tissue non specific) synthesis/secretion of alkaline phosphatase could be associated with decreased mineralization and ossification process in arthritis condition.