Disfunción temporomandibular en pacientes con síndrome de Sjögren / Temporomandibular dysfunction in patients with Sjögren's syndrome

Introducción: El síndrome de Sjögren es una enfermedad reumática, inflamatoria y crónica caracterizada por dolor articular y resequedad de las mucosas. La articulación temporomandibular (ATM) y las glándulas parótidas y salivales suelen estar afectadas. Objetivo: Determinar la prevalencia de la afectación de la articulación temporomandibular en pacientes con síndrome de Sjögren. Métodos: Estudio básico, no experimental, descriptivo y de corte transversal que incluyó a 78 pacientes con diagnóstico de síndrome de Sjögren atendidos en la Unidad Móvil número 2 de Alausi entre 2017 y 2019. Para identificar la afectación de la ATM se realizaron ecosonografías. Se usó la prueba de correlación de Pearson para establecer la relación entre las características de la enfermedad y la presencia de afectación articular. Resultados: El promedio de edad fue de 46,18 años. Predominaron los pacientes entre 40 y 60 años (62,82 por ciento), el sexo femenino (85,90 por ciento), y el tiempo de evolución de la enfermedad entre 3 y 5 años (50,00 por ciento). El 79,49 por ciento presentó síndrome de Sjögren secundario, y la artritis reumatoide fue la causa más frecuente (54,84 por ciento). El 65,38 por ciento tenía algún tipo de afectación de la ATM, y el hallazgo más común fue la disminución del espacio articular (56,86 por ciento). Conclusiones: Existió una elevada prevalencia de afectación de la ATM en los pacientes con síndrome de Sjögren, y el hallazgo principal fue la disminución del espacio articular. Se encontró una correlación positiva media entre el tiempo de evolución de la enfermedad y la presencia de afectación de la ATM(AU)

Introduction: Sjögren's syndrome is a rheumatic, inflammatory and chronic disease characterized by the presence of joint pain and dryness of the mucous membranes. The temporomandibular joint and the parotid and salivary glands usually have a high frequency of involvement. Objective: To determine the prevalence of temporomandibular involvement in patients with Sjögren's syndrome. Methods: Descriptive, correlational and explanatory study that included 78 patients diagnosed with Sjögren's syndrome treated in the mobile unit number 2 of Alausi. Echocardiography of the temporomandibular joints was performed to identify the involvement of the joint. Pearson's correlation test was used to establish a relationship between the characteristics of the disease and the presence of joint involvement. Results: Average age of 46.18 years with a predominance of patients between 40 and 60 years of age (62.82 percent), of the female sex (85.90 percent) and with time of evolution of the disease between 3 and 5 years (50.00 percent). 79.49 percent of the cases presented secondary Sjögren's syndrome, rheumatoid arthritis being the most frequent disease (54.84 percent). 65.38 percent of the patients presented some type of involvement of the temporomandibular joint, with the decrease in joint space being the most frequent finding (56.86 percent). Conclusions: There was a high prevalence of temporomandibular joint involvement in patients with Sjögren's syndrome, with the decrease in joint space being the finding that was most frequently identified. A positive average correlation was found between the time of evolution of the disease and the presence of temporomandibular joint involvement(AU)

Cambio de diagnóstico y de categoría diagnóstica en pacientes con artritis idiopática juvenil en 7 años de seguimiento / Change of diagnosis and diagnostic category in patients with juvenile idiopathic arthritis within 7 years of follow-up

Resumen: Introducción: Al menos 50% de los pacientes pediátricos portadores de artritis idiopática juvenil (AIJ) continuará control en reumatología adulto. La clasificación de la Liga Internacional de Asociaciones de Reumatología (ILAR) vigente, actualmente en revisión, difiere de la clasificación de las artritis inflamatorias del adulto. Se ha reportado cambios de categoría en 10,8% de los pacientes durante el seguimiento. Objetivo: Analizar los pacientes con AIJ seguidos al menos 7 años para objetivar cambios de diagnós tico en la transición, e identificar factores de mal pronóstico funcional. Pacientes y Método: Estudio retrospectivo en base a registros clínicos. Se incluyó a la totalidad de los pacientes con AIJ controla dos en policlínico pediátrico del Hospital de Puerto Montt entre el año 2005 y 2017, que cumplieron siete o más años de seguimiento. Se realizó análisis descriptivo en base a variables clínicas: categoría diagnóstica, tiempo de evolución al diagnóstico, actividad clínica y serológica, y tiempo de evolución al inicio de la terapia farmacológica. Resultados: Se evaluaron 18 pacientes, 3 Oligo-articular (OA) persistente, 1 OA extendida, 4 Poli-articular (PA) factor reumatoide (FR) negativo, 4 PA FR positivo, 5 Sistémicas, 1 Psoriática, todos con seguimiento mayor a 7 años. Once de 18 niños fueron transfe ridos a adultos. Tres de 11 cambiaron de diagnóstico a Artritis Reumatoide (AR) más otra enferme dad autoinmune: Síndrome de Sjögren + Lupus eritematoso sistémico, Púrpura trombocitopénico inmune, Enfermedad autoinmune no clasificada y cinco de 11 niños de categoría ILAR: OA a Artritis reumatoide juvenil, OA extendida a PA FR negativo, 3 Sistémicas a PA FR negativo. Edad de inicio, formas poli-articulares, retrasos en diagnóstico y comienzo de terapia se asociaron a secuelas e infla mación persistente. Conclusiones: Ocho de once pacientes transferidos cambiaron denominación diagnóstica y/o presentaron otras enfermedades autoinmunes. Algunos factores de mal pronóstico deben mejorar.

Abstract: Introduction: At least 50% of pediatric patients with Juvenile Idiopathic Arthritis (JIA) will require continued fo llow-up in adult rheumatology. The present International League of Associations for Rheumatology (ILAR) classification, currently under revision, differs from its classification of inflammatory arthritis in adults. Category changes have been reported in 10.8% of patients during follow-up. Objective: To analyze JIA patients in follow-up for at least 7 years to detect diagnosis changes during transition to adult care, identifying factors of poor functional prognosis. Patients and Method: Retrospective study based on medical records of JIA patients seen at the pediatric polyclinic of the Puerto Montt Hospital between 2005 and 2017, who were monitored for at least 7 years. Descriptive analysis was performed according to clinical variables: diagnostic category, evolution before diagnosis, clinical and serological activity, and evolution before starting drug therapy. Results: We evaluated 18 pa tients, corresponding to 3 patients with persistent oligoarticular arthritis (OA), 1 with extended OA, 4 with polyarticular arthritis (PA) rheumatoid factor (RF) negative, 4 with PA RF positive, 5 with syste mic JIA, and 1 with psoriatic arthritis, all have had follow-up more than 7 years. 11 out of 18 patients transitioned to adult care. Three out of 11 patients changed diagnosis to Rheumatoid Arthritis (RA) plus another autoimmune disease such as Sjögren's Syndrome + Systemic Lupus Erythematosus, Immune thrombocytopenia, or unclassified autoimmune disease, and 5 out of 11 children changed ILAR category from OA to Juvenile Rheumatoid Arthritis, extended OA to PA RF negative, and 3 from Systemic arthritis to PA RF negative. Age of onset, polyarticular forms, delay in diagnosis, and the start of therapy were associated with sequelae and persistent inflammation. Conclusions: Eight of the eleven JIA patients who transitioned to adult care changed their diagnosis or presented other autoimmune diseases. Some factors of poor prognosis must improve.

Relación entre adiposidad corporal y presión arterial en niños y adolescentes con enfermedades reumáticas / Relationship between body adiposity and blood pressure in children and adolescents with rheumatic diseases

Introducción: las enfermedades reumáticas son un grupo de afecciones que se caracterizan por la presencia, generalmente, de dolor, inflamación, deformidad, discapacidad funcional y disminución de la percepción de calidad de vida relacionada con la salud. Las complicaciones cardiovasculares son frecuentes en el curso de estas enfermedades; los trastornos nutricionales pueden ser causa o consecuencia de estas complicaciones. Objetivo: determinar la relación existente entre adiposidad corporal y presión arterial en niños y adolescentes con enfermedades reumáticas. Metodos: estudio descriptivo, de corte transversal en 45 pacientes con enfermedades reumáticas diagnosticadas según los criterios del American College of Reumathology. Se utilizó la técnica de bioimpedancia para determinar el porcentaje de grasa corporal e índice de correlación de Pearson para determinar relación entre variable. Resultados: promedio de edad de 12,26 ± 8,74 años, predominio de pacientes femeninas (68,69 por ciento) y entre 15 y 18 años de edad (42,22 por ciento). La enfermedad reumática más representada fue la artritis idiopática juvenil (46,67 por ciento). Predominaron los pacientes con aumento del porcentaje de grasa, donde el sobrepeso (44,44 por ciento) y la obesidad (20,0 por ciento) fueron los más representados. El 20,0 por ciento de los pacientes incluidos en la investigación presentaron alteraciones de la presión arterial con predominio en pacientes obesos (33,33 por ciento) y con sobrepeso (20,0 por ciento). Conclusiones: Mantener un adecuado control de la adiposidad corporal disminuye considerablemente la posibilidad de aparición de trastornos de la presión arterial. La bioimpedancia eléctrica es un método de fácil aplicación y de comprobada efectividad para determinar el porcentaje de grasa(AU)

Introduction: rheumatic diseases are a group of conditions that are characterized by the presence, usually, of pain, inflammation, deformity, functional disability and decreased perception of quality of life related to health. Cardiovascular complications are frequent in the course of these diseases; Nutritional disorders may be the cause or consequence of these complications. Objective: to determine the relationship between body adiposity and blood pressure in children and adolescents with rheumatic diseases. Methodology: descriptive, cross-sectional study in 45 patients with rheumatic diseases diagnosed according to the criteria of the American College of Reumathology. The bioimpedance technique was used to determine the percentage of body fat and Pearson's correlation index to determine the relationship between variables. Results: average age of 12.26 ± 8.74 years, predominance of female patients (68.69 percent) and between 15 and 18 years of age (42.22 percent). The most represented rheumatic disease was juvenile idiopathic arthritis (46.67 percent). Patients with increased fat percentage predominated, where overweight (44.44 percent) and obesity (20.0 percent) were the most represented. 20.0 percent of the patients included in the investigation presented alterations in blood pressure, predominantly in obese patients (33.33 percent) and overweight (20.0 percent). Conclusions: Maintaining adequate control of body adiposity considerably reduces the possibility of the appearance of blood pressure disorders. The electric bioimpedance is a method of easy application and proven effectiveness to determine the percentage of fat(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.

Correlation of ultrasonography synovitis with disease activity and clinical response to etanercept treatment in juvenile idiopathic arthritis patients

This study aimed to investigate the correlation of ultrasonography (US) of synovitis with disease activity and clinical response to etanercept (ETN) in juvenile idiopathic arthritis (JIA) patients. Eighty-two JIA patients who underwent ETN treatment for 24 weeks were consecutively enrolled. US evaluations of 28 joints (shoulder, elbow, wrist, metacarpophalangeal, and proximal interphalangeal of hands and knee) at baseline were performed using grey-scale US and power doppler (PD) US, and US synovitis was defined as grey-scale abnormalities or PD abnormalities. Clinical response was assessed according to the ACRpedi 50 response criteria. In total, 2296 joints were scanned and 608 (26.5%) joints presented US synovitis, which was numerically higher than clinical synovitis (513 (22.3%)). The mean number of joints showing synovitis on US was 7.42±3.35, which was also numerically higher than that of clinical synovitis (6.26±2.70). The number of joints showing synovitis on US was positively correlated with C-reactive protein, erythrocyte sedimentation rate, number of joints with active disease, number of joints with limited range of motion, physician's global assessment of disease activity, parent/patient global assessment of overall well-being, and childhood health assessment questionnaire score. Most interestingly, the baseline number of joints showing synovitis on US was increased in ACRpedi 50 response JIA patients compared to non-response JIA patients, and it serves as an independent predictive factor for higher clinical response to ETN treatment. In conclusion, US is a more sensitive test to evaluate subclinical synovitis and disease activity in JIA patients, and US synovitis might serve as a marker for predicting increased clinical response rate to ETN treatment.

Trastornos temporomandibulares en la infancia: bruxismo de sueño y compromiso de atm en artritis idiopática juvenil / Temporomandibular disorders in childhood: sleep bruxism and temporomandibular joint involvement in juvenile idiopathic arthritis

Los trastornos temporomandibulares (TTM) corresponden a un grupo de condiciones musculoesqueletales y neruromusculares que involucran las articulaciones temporomandibulares (ATM), los músculos masticatorios y todos los tejidos asociados. La etiología de los TTM es considerada multifactorial, siendo el bruxismo de sueño (BS) uno de muchos factores asociados con TTM dolorosos. Tanto los TTM como el BS se presentan en adultos y niños y actualmente es sabido que la etiopatogenia de ambos no difiere de acuerdo a la edad. Las ATM son articulaciones sinoviales que pueden verse afectadas por diversos TTM o por condiciones sistémicas como la artritis idiopática juvenil (AIJ). La ATM está involucrada en un 40% de los pacientes con AIJ, siendo subestimada debido a que clínica-mente se manifiesta con poco dolor. En el presente artículo se revisarán los conceptos de TTM y BS en niños, así como también la manifestación de la AIJ en el territorio orofacial, entregando una aproximación de su etiopatogenia, identificación y manejo.

Temporomandibular disorders (TMD) encompass a group of musculoskeletal and neuromuscular conditions that involve the temporomandibular joints (TMJ), the masticatory muscles, and all associated tissues. TMD's etiology is considered to be mul-tifactorial, were sleep bruxism (SB) is one of many causes of painful TMD. TMD and SB can present in adults and children and the etiology does not differ regarding age.TMJ are synovial joints that can be affected by many TMD as well as systemic conditions such as juvenile idiopathic arthritis (JIA). TMJ are involved in 40% of patients with JIA, which is usually underestimated because of its painless presentation.This article will review the concepts of TMD and SB in children, as well as JIA presentation in the orofacial region.

Temporomandibular joint disorders as the only manifestation of juvenile idiopathic arthritis: a case report / Comprometimento da articulação temporomandibular como única manifestação da artrite idiopática juvenil: um relato de caso

ABSTRACT Juvenile idiopathic arthritis is a term used to include all chronic childhood arthritis of unknown etiology. It is characterized by chronic inflammation persisting for at least 6 weeks, beginning before 16 years of age. The characteristics present are chronic synovitis, arthralgia, impaired joint mobility in at least one joint, and erosion with destruction of cartilage and subchondral bone, that could be associated or not with systemic involvement, according to each subtype of the disease. During the pathologic process, the temporomandibular joint can be involved by the juvenile idiopathic arthritis, resulting in severe mandibular dysfunction, with higher frequency in female patients. Initially, these lesions can show minor alterations like flattening of the condyle, erosions, and evolve to severe lesions, like destruction of the head of the condyle. We report a case of male patient who had destruction of both condyles, as a result from juvenile idiopathic arthritis. Proposed mechanisms to explain the juvenile idiopathic arthritis was reviewed. In this report the patient did not have pain or inflammatory process, and the temporomandibular diseases was the only manifestation.

RESUMO Artrite idiopática juvenil é um termo usado para incluir toda artrite infantil crônica de etiologia desconhecida. É caracterizada por uma inflamação crônica, que persiste por pelo menos 6 semanas, com início antes dos 16 anos de idade. As características presentes são sinovite crônica, artralgia, mobilidade articular diminuída em pelo menos uma articulação, e erosão com destruição da cartilagem e do osso subcondral, podendo ser associada ou não com o envolvimento sistêmico, de acordo com cada subtipo da doença. Durante o processo patológico, a articulação temporomandibular pode ser envolvida pela artrite idiopática juvenil, resultando em disfunção mandibular severa, com maior frequência em pacientes do sexo feminino. Inicialmente, estas lesões podem mostrar pequenas alterações, como achatamento do côndilo e erosões, e evoluir para lesões graves, como a destruição da cabeça do côndilo. Relatou-se o caso de um paciente do sexo masculino, que apresentou destruição de ambos os côndilos, como resultado da artrite idiopática juvenil. Os mecanismos para explicar a artrite idiopática juvenil foram revisados na literatura. Neste relato de caso, o paciente não apresentou dor e nem processo inflamatório, sendo o comprometimento da articulação temporomandibular a única manifestação.

Latent tuberculosis infection screening in juvenile idiopathic arthritis patients preceding anti-TNF therapy in a tuberculosis high-risk country / Rastreamento da infecção latente por tuberculose em pacientes com artrite idiopática juvenil previamente à terapia anti-TNF em um país de alto risco para tuberculose

Abstract Objectives: To evaluate, in an endemic country, the long-term efficacy of latent tuberculosis infection (LTBI) screening and primary prophylaxis in patients with JIA receiving TNF blockers. Methods: This was a retrospective cohort that included JIA patients eligible to anti-TNF therapy. Patients were screened for LTBI prior to anti-TNF using tuberculin skin test (TST), chest X-ray and history of exposure to TB. Subjects were regularly followed at 2-month intervals. Results: Sixty-nine JIA patients with current age of 17.4 ± 5.8 years, mean disease duration of 5.0 ± 4.9 years were included. Forty-seven patients received a single anti-TNF, while 22 patients switched to another anti-TNF once or twice: 57 were treated with etanercepte, 33 patients with adalimumab and 3 infliximab. LTBI screening was positive in three patients: one had TST-positive and history of TB exposure and two had solely TST-positive. No active TB was diagnosed during the study period (median of follow-up was 3.8 years). Conclusion: Long-term evaluation revealed that LTBI screening and primary prophylaxis before anti-TNF treatment was effective in a high-risk country and TST was the most sensitive parameter to identify these patients.

Resumo Objetivo: Avaliar, em um país endêmico, a eficácia em longo prazo do rastreamento à procura de infecção latente por tuberculose (ILTB) e profilaxia primária em pacientes com AIJ em uso de bloqueadores do TNF. Métodos: Trata-se de uma coorte retrospectiva que incluiu pacientes com AIJ elegíveis para a terapia anti-TNF. Os pacientes foram rastreados à procura de ILTB previamente ao uso de anti-TNF por meio do teste tuberculínico (TT), radiografia de tórax e história de exposição à TB. Os indivíduos foram acompanhados regularmente em intervalos de dois meses. Resultados: Incluíram-se 69 pacientes com AIJ com idade atual de 17,4 ± 5,8 anos, com média de duração da doença de 5 ± 4,9 anos; 47 pacientes receberam um único anti-TNF, enquanto 22 foram transferidos para outro anti-TNF uma ou duas vezes: 57 foram tratados com etanercepte, 33 com adalimumabe e três com infliximabe. O rastreamento à procura de ILTB foi positivo em três pacientes: um era TT positivo e tinha história de exposição à TB e dois apenas eram TT positivo. Não foi diagnosticado caso de TB ativa durante o período de estudo (mediana de seguimento de 3,8 anos). Conclusão: A avaliação em longo prazo revelou que o rastreamento à procura de ILTB e a profilaxia primária antes do tratamento com anti-TNF foram eficazes em um país de alto risco para TB e o TT foi o parâmetro mais sensível para identificar esses pacientes.

A fatal case of tuberculous meningitis in a child with juvenile idiopathic arthritis: a diagnostic challenge

Abstract The prognosis of tuberculous meningitis, a rare form of extrapulmonary tuberculosis, depends on the stage of treatment initiation. We report a fatal case of tuberculous meningitis. The patient had received successive tumor necrosis factor (TNF) antagonists and abatacept to treat juvenile idiopathic arthritis, with negative results for polymerase chain reaction and acid-fast bacilli on smear, had normal cerebrospinal fluid (CSF) adenosine deaminase and glucose levels. Six weeks post-admission, the CSF culture demonstrated Mycobacterium tuberculosis. The altered immunological responses caused by anti-TNF treatment made the diagnosis challenging. Clinicians should bear this in mind and, if suspected, treatment should be initiated immediately.

La artritis reumatoidea juvenil y su influencia en la alteración de desarrollo de la mandíbula / Juvenile rheumatoid arthritis and its influence on the alteration of jaw development

La artritis reumatoidea juvenil (ARJ) es una enfermedad inflamatoria autoinmune que se presenta en niños menores de 16 años. Es de curso crónico, etiología desconocida, y afecta sobre todo las articulaciones, como la temporomandibular (ATM). El daño de la ATM puede ocasionar: alteraciones en el crecimiento facial (micrognatia), maloclusión clase II, mordida abierta anterior, desviaciones laterales, erosiones óseas, destrucción del cón-dilo, oclusión disfuncional y alteración de la estética facial, entre otras consecuencias. La posición oclusal neurofisiológica lograda por medio de elementos electrónicos, como el Transcutaneus Electrical Neural Stimulation (TENS), y mantenida por el Dispositivo Intaroral (DIO) podría posibilitar la remodelación de la cabeza del cóndilo, en pacientes en crecimiento, en los que la enfermedad se halla controlada, regulando así también la sintomatología dolorosa...

A Artrite Idiopática Juvenil como fator de risco para a erosão dentária: estudo preliminar / The Juvenile Idiopathic Arthritis as a risk factor for dental erosion: a preliminary study

O objetivo deste estudo foi avaliar se a presença da artrite idiopática juvenil (AIJ) em crianças e adolescentes pode ser considerada como indicador de risco para a erosão dentária. Para tal, foram utilizadas oitenta crianças e adolescentes com diferentes tipos de AIJ e 23 pacientes saudáveis como controle. Os critérios de inclusão foram diagnóstico de AIJ e classificação do subtipo de acordo com os critérios da Liga Internacional das Associações de Reumatologia(ILAR). Os dados sobre variáveis nutricionais, fatores socioeconômicos, vômitos, ingestão de medicamentos, hábitos alimentares e orais foram coletados por meio de questionário estruturado.Todos os pacientes foram submetidos a medição do fluxo salivar e exame clínico usando o índice Exame Básico de Desgaste Erosivo (BEWE). Dezesseis pacientes, todos afetados pelo subtipo oligo articular de AIJ mostraram redução da quantidade de saliva estimulada; dois deles apresentaram erosão e relataram consumo diário de refrigerantes. Foi relatado vômitos (uma vez por dia) por nove pacientes devido à ingestão de metotrexato sem correlação com o subtipo de AIJ. Nenhum deles exibiu erosão. O indicador de risco para a erosão dentária foi o subtipo de AIJ (artrite oligo articular), não havendo correlação significativa da presença de erosão dentária com as variáveis nutricionais, ingestão de medicamentos, hábitos alimentares e orais.

The purpose of this study was evaluate the existence of prevalence and risk indicators of dental erosion in patients affected by juvenile idiopathic arthritis (JIA). Eighty patients (61 females and 19males) with different kinds of JIA and 23 healthy control ones aging from 3 to 19 years were included in the study. The inclusion criteria were diagnosis of JIA and subtype classification according to the International League of Associations for Rheumatology (ILAR) criteria. Data on nutrition al variables, socioeconomic factors, vomiting, drug intake and dietary and oral habits were collected using a questionnaire. All patients underwent saliva measurement and clinical examination using Basic Erosive Wear Examination (BEWE) Index. Sixteen patients, all affected by the oligo articular subtype of JIA showed a reduced quantity of stimulated saliva; two of them showed erosion andre ported a daily soft drink intake. Vomiting (once a day) was reported by nine patients and was dueto methotrexate intake with no correlation with JIR subtype. None of them exhibited erosion. Risk indicator for wear was JIA subtype (oligo articular arthritis). There was no significant correlation of the presence of dental erosion with nutritional variables, drug intake or dietary and oral habits.

Problemas autoinmunes como factor etiológico de las alteraciones del desarrollo de la mandíbula: artritis reumatoidea juvenil / Autoimmune problems as etiolocial factors of developmental disorders of the jaw: juvenile idiopathic arthritis

La artritis reumatoidea juvenil (ARJ) es una enfermedad inflamatoria autoinmune en niños menores de 16 años. Es de curso crónico, etiología desconocida y afecta sobre todo las articulaciones, como la temporomandibular (ATM). El compromiso de la ATM puede ocasionar alteraciones en el crecimiento facial (micrognatia), maloclusión clase II, mordida abierta anterior, desviaciones laterales, erosiones óseas, destrucción del cóndilo, oclusión disfuncional y alteración de la estética facial, entre otras consecuencias. La posición oclusal neurofisiológica lograda por medio de elementos electrónicos, como el Transcutaneous Electrical Neural Stimulation (TENS), y mantenida por el dispositivo intraoral (DIO), posibilitaría la remodelación de la cabeza del cóndilo, en pacientes en crecimiento, en los que la enfermedad se halla controlada, controlando así también la sintomatología dolorosa.

Síndrome de ativação macrofágica em paciente com artrite idiopática juvenil sistêmica / Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis

A síndrome de ativação macrofágica (SAM) é uma doença rara e potencialmente fatal, normalmente associada às doenças reumáticas crônicas, em especial a artrite idiopática juvenil. É incluída no grupo das formas secundárias de síndrome hemofagocítica, cujas outras causas podem ser as doenças linfoproliferativas e infecções. As manifestações clínicas e laboratoriais mais importantes são a febre não remitente, esplenomegalia, hemorragias, disfunção hepática, citopenias, hipoalbuminemia, hipertrigliceridemia e hiperferritinemia. O tratamento deve ser iniciado rapidamente, e a maioria dos casos responde bem aos corticosteroides e à ciclosporina (CSA). O vírus Epstein-Barr (EBV) é descrito como possível gatilho para muitos casos de SAM, especialmente naqueles em tratamento com bloqueadores do fator de necrose tumoral (TNF). Nos casos refratários ao tratamento convencional, etoposide (VP16) deve ser administrado, em associação com corticosteroides e CSA. Nosso objetivo foi descrever um caso raro de síndrome hematofagocítica provavelmente secundária à infecção pelo vírus Epstein-Barr (EBV), em paciente com artrite idiopática juvenil sistêmica, confirmada pelas manifestações clínicas e laboratoriais típicas, mielograma e sorologia positiva contra o EBV, que atingiu remissão completa após inclusão no protocolo de tratamento HLH-04.

Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein–Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemiconset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.

Neurite óptica em paciente com artrite idiopática juvenil / Optic neuritis in juvenile idiopathic arthritis patient

O bloqueio do TNF tem tido sucesso no tratamento de algumas doenças reumáticas, como a espondiloartrite. Relatam-se muitas complicações infecciosas com a terapia anti-TNF, principalmente infecções bacterianas, micobacterianas, virais e fúngicas. A Entamoeba histolytica é um protozoário extracelular que causa principalmente colite e abscesso hepático, sendo que a perfuração intestinal é uma complicação rara, com alta mortalidade. O TNF é considerado o principal mediador da imunidade celular contra a amebíase. Inicialmente, é quimiotático para a E. histolytica, potencializando sua adesão ao enterócito por meio da lectina galactose-inibível, e depois ativando os macrófagos para matarem a ameba pela liberação de NO; assim, o bloqueio do TNF poderia ser prejudicial, aumentando a virulência amebiana. Descreve-se o caso de uma mulher de 46 anos com espondiloartrite que apresentou uma perfuração do colo por colite amebiana invasiva durante uso de anti-TNF.

Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required.

Associação entre artrite idiopática juvenil e osteogenesis imperfecta: relato de caso / Association between Juvenile idiopathic arthritis and osteogenesis imperfecta: case report

Os autores relatam o caso de uma paciente de 53 anos que apresenta uma rara associação entre artrite idiopática juvenil (AIJ) e osteogenesis imperfecta (OI), com acometimento poliarticular, incluindo a articulação temporomandibular. Apresentam uma revisão da literatura e uma discussão dos aspectos radiológicos do acometimento da referida articulação. Não foram encontrados relatos de casos com semelhante associação de doenças na literatura especializada.

The authors report a rare association case of juvenile idiopathic arthritis (JIA) and osteogenesis imperfecta (OI) in a 53 years-old female patient, present a literature review and discuss the radiological aspects of the temporo-mandibular joint involvement. To our knowledge, this is the first case report of JIA an OI association.

Pediatric uveitis: an update

Because of their varied spectrum of clinical presentation and difficulty in management, pediatric uveitis remains a challenge to the ophthalmologist. Variations in clinical presentation, difficulties in eye examination, extended burden of the inflammation over quality of life, limited treatment modalities, risk of amblyopia are the main challenges in the management of pediatric uveitis. Pediatric uveitis is a cause of significant ocular morbidity and severe vision loss is found in 25-33% of such cases. This article summarizes the common causes of uveitis in children with special approach to the evaluation and diagnosis of each clinical entity

Manifestaciones respiratorias de las enfermedades reumáticas en niños / Respiratory manifestation of rheumatic disease in children

Rheumatic diseases are a heterogeneous group of autoimmune basis and mainly joint and skin manifestations. Respiratory manifestations of these are less common in children than in adults, can affect all respiratory structures, however the most common are the pleural space with pleuritis and effusion and interstitial lung disease. Alterations are usually subclinical beginning, which are evident to the tomography of chest or lung function tests as spirometry or diffusion test for carbon monoxide. Some cases presents with severe respiratory impairment as respiratory bleeding or pneumonia. The comprehensive evaluation included clinical history, imaging studies and pulmonary function is key in the evaluation and management of these patients.

Las enfermedades reumáticas son un grupo heterogéneo de patologías de base autoinmune y manifestaciones principalmente articulares y cutáneas. Las expresiones respiratorias de estas en niños son menos frecuentes que en los adultos, pueden afectar todas las estructuras respiratorias, sin embargo las más frecuentes son el espacio pleural con pleuritis y derrame y la afectación intersticial pulmonar. Las alteraciones suelen ser de comienzo subclínico, que se hacen evidentes a la exploración con tomografía de tórax o en pruebas de función respiratoria como espirometría o prueba de difusión de monóxido de carbono. Algunos casos cursan con afectación respiratoria severa, como hemorragia respiratoria o neumonías sobre agregadas. La evaluación integral con historia clínica, estudios de imágenes y de función respiratoria son la clave en la evaluación y manejo de estos pacientes.

Alterações da articulação temporomandibular e suas repercussões orofaciais em pacientes portadores de artrite idiopática juvenil / Temporomandibular joint alterations and their orofacial complications in patients with juvenile idiopathic arthritis

Os pacientes com artrite idiopática juvenil (AIJ) podem apresentar alterações no metabolismo ósseo e no crescimento esqueletal, além de danos na articulação temporomandibular (ATM), o que pode gerar alterações extraorais e/ou intraorais, acarretando distúrbios craniofaciais. Nosso objetivo é promover uma revisão da literatura sobre as alterações orofaciais em pacientes com AIJ. Dentre os distúrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular, geradas por disfunções na região da ATM, parecem de grande prevalência nesses pacientes. As alterações mais encontradas são: retrognatismo, micrognatia, mordida aberta anterior, apinhamento dentário, assimetria facial e limitação de abertura de boca. Assim, o reumatologista torna-se uma peça-chave na detecção precoce dessas disfunções, ajudando no encaminhamento do paciente ao dentista. O diagnóstico, por sua vez, deve ser realizado pelo ortodontista, utilizando exames clínico e de imagem, o que possibilita tratamento precoce e prognóstico favorável. Distúrbios na ATM devem ser tratados de forma multidisciplinar, incluindo tratamento farmacológico para o controle da dor e tratamento odontológico por meio de aparelhagem funcional e/ou ortodôntica, fisioterapêutico e por vezes fonoaudiológico. Concluímos que dentre os distúrbios orofaciais em pacientes com AIJ, as mudanças no crescimento mandibular geradas por disfunções na região da ATM parecem de grande prevalência. Tais disfunções podem promover, principalmente, mordida aberta, retrusão mandibular, micrognatia, apinhamento dentário e assimetria facial. O reumatologista pode detectar tais alterações precocemente, encaminhando o paciente de imediato a uma equipe que deve ser, preferencialmente, multidisciplinar, composta por ortodontista, fisioterapeuta e fonoaudiólogo, visando reduzir futuras complicações oclusais e de crescimento mandibular.

Patients with juvenile idiopathic arthritis (JIA) can have alterations in bone metabolism and skeletal growth, as well as damage to the temporomandibular joint (TMJ), which can generate extra and/or intraoral alterations, resulting in craniofacial disorders. Our goal is to carry out a review of the literature on orofacial alterations in patients with JIA. Among the orofacial disorders in patients with JIA, alterations in mandibular growth, caused by dysfunctions in the TMJ region, seem highly prevalent in these patients. The most often found alterations are: retrognathia, micrognathia, anterior open bite, dental crowding, facial asymmetry and mouth opening limitation. Thus, the rheumatologist becomes a key agent in the early detection of these disorders, helping with patient referral to a dentist. The diagnosis, in turn, should be performed by the orthodontist, using clinical examination and imaging methods, allowing early treatment and a favorable prognosis. TMJ disorders should be treated by a multidisciplinary team, including pharmacological treatment for pain control and dental care through functional appliance and/or orthodontic therapy, physical therapy and sometimes, speech therapy. We conclude that among the orofacial disorders in patients with JIA, alterations in mandibular growth generated by dysfunctions in the TMJ region seem highly prevalent. Such dysfunctions can cause mainly open bite, mandibular retrusion, micrognathia, dental crowding and facial asymmetry. The rheumatologist can detect these alterations at an early stage, with immediate patient referral to a team that should preferably be a multidisciplinary one, consisting of an orthodontist, physical therapist and speech therapist, to reduce future occlusal and mandibular growth complications.