Clinical characteristics and diagnostic indicators of macrophage activation syndrome in patients with systemic lupus erythematosus and adult-onset Still's disease / 北京大学学报(医学版)

OBJECTIVE@#To analyze and compare the clinical and laboratory characteristics of macrophage activation syndrome (MAS) in patients with systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and to evaluate the applicability of the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for MAS complicating systemic juvenile idiopathic arthritis (sJIA) in different auto-immune diseases contexts and to propose new diagnostic predictive indicators.@*METHODS@#A retrospective analysis was conducted on the clinical and laboratory data of 24 SLE patients with MAS (SLE-MAS) and 24 AOSD patients with MAS (AOSD-MAS) who were hospitalized at Peking University People's Hospital between 2000 and 2018. Age- and sex-matched SLE (50 patients) and AOSD (50 patients) diagnosed in the same period without MAS episodes were selected as controls. The cutoff values for laboratory indicators predicting SLE-MAS and AOSD-MAS were determined using receiver operating characteristic (ROC) curves. Furthermore, the laboratory diagnostic predictive values for AOSD-MAS were used to improve the classification criteria for systemic juvenile idiopathic arthritis-associated MAS (sJIA-MAS), and the applicability of the revised criteria for AOSD-MAS was explored.@*RESULTS@#Approximately 60% of SLE-MAS and 40% of AOSD-MAS occurred within three months after the diagnosis of the underlying diseases. The most frequent clinical feature was fever. In addition to the indicators mentioned in the diagnosis criteria for hemophagocytic syndrome revised by the International Society for Stem Cell Research, the MAS patients also exhibited significantly elevated levels of aspartate aminotransferase and lactate dehydrogenase, along with a significant decrease in albumin. Hemophagocytosis was observed in only about half of the MAS patients. ROC curve analysis demonstrated that the optimal discriminative values for diagnosing MAS was achieved when SLE patients had ferritin level≥1 010 μg/L and lactate dehydroge-nase levels≥359 U/L, while AOSD patients had fibrinogen levels≤225.5 mg/dL and triglyceride levels≥2.0 mmol/L. Applying the 2016 sJIA-MAS classification criteria to AOSD-MAS yielded a diagnostic sensitivity of 100% and specificity of 62%. By replacing the less specific markers ferritin and fibrinogen in the 2016 sJIA-MAS classification criteria with new cutoff values, the revised criteria for classifying AOSD-MAS had a notable increased specificity of 86%.@*CONCLUSION@#Secondary MAS commonly occurs in the early stages following the diagnosis of SLE and AOSD. There are notable variations in laboratory indicators among different underlying diseases, which may lead to misdiagnosis or missed diagnosis when using uniform classification criteria for MAS. The 2016 sJIA-MAS classification criteria exhibit high sensitivity but low specificity in diagnosing AOSD-MAS. Modification of the criteria can enhance its specificity.

Enfermedad de Scheuermann lumbar atípica en rara asociación con artritis idiopática juvenil / Atypical lumbar Scheuermann disease and juvenile idiopathic arthritis: a rarely association

El dolor lumbar en los adolescentes es causa frecuente de motivo de consulta en reumatología y obedece a diferentes causas. Se presenta un caso clínico de un adolescente de 14 años de edad, de procedencia rural que acudió a consulta refiriendo dolor y aumento de volumen de ambas rodillas de 3 meses de evolución, acompañado de dolor lumbar desde hacía más de 2 años y que había requerido tratamiento con antinflamatorios no esteroideos y reposo, sin otros síntomas sistémicos acompañantes. Al examen físico se encontró artritis de rodillas, aumento de la cifosis fisiológica en la columna dorsal y puntos sacroilíacos positivos. En los exámenes complementarios fue significativa la presencia del HLA-B27, sinovitis en bolsa subcuadricipital bilateral detectada mediante ultrasonido de rodillas, así como hallazgos en las radiografías a nivel de los cuerpos de las vértebras lumbares característicos de la enfermedad de Scheuermann, y esclerosis de ambas sacroilíacas, características de artritis idiopática juvenil. Se concluyó que el paciente padecía de dos afecciones que por mecanismos diferentes causan dolor lumbar(AU)

Low back pain in adolescents is a frequent reason for consultation in rheumatology and is due to different causes. A clinical case of a 14-year-old adolescent from rural origin who comes to the clinic reporting pain and volume increase in both knees of three months of evolution accompanied by low back pain of more than two years of evolution that had required treatment is presented. with non-steroidal anti-inflammatory drugs and rest, without other accompanying systemic symptoms, physical examination revealed knee arthritis, increased physiological kyphosis in the thoracic spine and positive sacroiliac points. In the complementary tests, the presence of HLA-B27, synovitis in the bilateral sub quadriceps bursa on ultrasound of the knees, findings in the radiographs at the level of the bodies of the lumbar vertebrae characteristic of Scheuermann's disease, and sclerosis of both sacroiliacs' characteristic of juvenile idiopathic arthritis, it is concluded that the patient suffers from two conditions, which by different mechanisms cause low back pain(AU)

Afectación articular en adolescente con artritis idiopática juvenil / Joint involvement in an adolescent with juvenile idiopathic arthritis

La artritis idiopática juvenil es una enfermedad inflamatoria sistémica y crónica que se caracteriza por el daño articular y la presencia de manifestaciones extraarticulares que afectan distintos órganos y sistemas de órganos del cuerpo humano. Como enfermedad tiene varias formas clínicas de presentación que se corresponden con posibles enfermedades en la edad adulta. El objetivo de la presente investigación es presentar el caso de un adolescente de 14 años de edad con historia de cuadro inflamatorio poliarticular de más de 3 años de duración con deformidad articular en ambas rodillas, lo cual es poco frecuente y que es expresión del proceso inflamatorio mantenido. Después del tratamiento fue dado de alta con una mejoría notable de los rangos de movimiento articular. En la actualidad evoluciona satisfactoriamente y lleva alrededor de un año en seguimiento en consulta externa sin exacerbaciones de la actividad clínica de la enfermedad. Se considera importante el reporte del caso para concientizar a la comunidad médica en relación con el diagnóstico precoz de esta enfermedad para minimizar el riesgo de aparición de complicaciones articulares y sistémicas(AU)

Juvenile idiopathic arthritis is a systemic and chronic inflammatory disease characterized by joint involvement and the presence of extra-articular manifestations that occur in different organs and organ systems of the human body. As a disease, it includes a series of clinical forms of presentation that correspond to possible diseases in adulthood. The objective of this research is to present the case of a 14-year-old adolescent with a history of polyarticular inflammatory symptoms lasting more than three years with the presence of rare joint deformity in both knees, which is an expression of the sustained inflammatory process. The case report is considered important to raise awareness in the medical community regarding the early diagnosis of this disease to minimize the risk of the appearance of joint and systemic complications(AU)

Niveles de ferritina en artritis idiopática juvenil de inicio sistémico comparada con otras fiebres de origen desconocido: estudio multicéntrico de pruebas diagnósticas / Ferritin levels in children with juvenile idiopathic arthritis of systemic onset and children with other causes of fever of unknown origin: A multicenter study of diagnostic tests

Resumen | Introducción. No se dispone de pruebas sensibles ni específicas para diagnosticar la artritis idiopática juvenil sistémica. Objetivo. Evaluar la utilidad diagnóstica de niveles de ferritina total cinco veces por encima del valor normal (ferritina total>5N) y el porcentaje disminuido (menor de o igual a 20 % de la ferritina total) de la ferritina glucosilada (ferritina glucosilada<20 %) para el diagnóstico de artritis idiopática juvenil sistémica en pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica. Materiales y métodos. Se hizo un estudio observacional de pruebas diagnósticas de corte transversal en menores de 16 años hospitalizados entre el 2010 y el 2014. El patrón diagnóstico de referencia fue el cumplimiento de los criterios de clasificación o diagnóstico confirmado en el seguimiento. Se determinaron las medidas de utilidad de las pruebas. Resultados. Se incluyeron 40 pacientes con fiebre de origen desconocido: 11 con artritis idiopática juvenil sistémica y 29 con otros diagnósticos. La mediana de la ferritina total fue mayor en la artritis idiopática juvenil sistémica (3.992 ng/ml) comparada con otras causas de fiebre de origen desconocido (155 ng/ml) (p=0,0027), así como la ferritina total>5N (90,91 % Vs. 51,72 %) (p=0,023). El porcentaje de ferritina glucosilada≤20 % fue de 96,5 % en otras fiebres de origen desconocido en comparación con la artritis idiopática juvenil sistémica (81,8 %) (p=0,178). La ferritina total>5N tuvo una sensibilidad del 91 %, una especificidad del 48 %; un cociente de probabilidades (Likelihood Ratio, LR) positivo de 1,76 y uno negativo de 0,19, demostrando mayor utilidad para el diagnóstico que la combinación de la ferritina total>5N y ferritina glucosilada≤20 %, cuya sensibilidad fue del 81,8 %, la especificidad del 48,3 %, un cociente de probabilidades LR positivo de 1,58 y un LR negativo de 0,38. Conclusión. En pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica, la ferritina total>5N demostró ser útil como prueba de tamización para el diagnóstico de artritis idiopática juvenil sistémica.

Abstract | Introduction: There are no sensitive or specific tests available to diagnose systemic juvenile idiopathic arthritis (sJIA). Objective: To assess the utility as diagnostic tests of total ferritin (TF) levels greater than 5 times the normal value (TF>5N) and the decreased percentage (less than or equal to 20% of TF) of glycosylated ferritin (GF≤20%) for the diagnosis of sJIA in patients with fever of unknown origin evaluated by pediatric rheumatology. Materials and methods: We conducted an observational, cross-sectional study of diagnostic tests in children under 16 years of age hospitalized between 2010 and 2014. The reference diagnostic standard was the fulfillment of the classification criteria or confirmed diagnosis at follow-up. We determined the measures of utility of the tests. Results: We included 40 patients with fever of unknown origin, 11 with sJIA, and 29 with other diagnoses. The median TF was higher in sAIJ (3992 ng/ml) versus other causes of fever of unknown origin (155 ng/ml) (p=0.0027), as well as TF>5N (90.91% versus 51.72%) (p=0.023). The percentage of GF≤20% was higher in patients with other causes of fever of unknown origin (96.5%) compared to sJIA (81.8%) (p=0.178). TF>5N had a sensitivity of 91%, specificity of 48%, positive likelihood ratio (LR) of 1.76, and negative LR of 0.19 demonstrating greater utility for the diagnosis of sJIA than the combination of FT> 5N with GFR <20%, with a sensitivity of 81.8%, specificity of 48.3%, positive LR of 1.58, and negative LR of 0.38. Conclusion: In patients with FUO evaluated by pediatric rheumatology, TF> 5N proved useful as a screening test for the diagnosis of sJIA.

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.

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.

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.

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)

Macrophage activation syndrome in a patient with systemic-onset juvenile idiopathic arthritis initially treated for atypical Kawasaki disease: a case-based review

Systemic-onset juvenile idiopathic arthritis [SoJIA] is the most common rheumatic disorder in children and its presentation can mimic atypical Kawasaki disease. The diagnosis of SoJIA is often challenging and children are often diagnosed and treated for Kawasaki disease initially, especially after an unremitting fever lasting for several days. This fact can delay the treatment of SoJIA as incorrect treatment with intravenous immunoglobulins [IVIG] is being given and this may probably lead to a worse outcome in those individuals. This is a case of a 12-month-old infant who was initially treated for atypical Kawasaki instead of a SoJIA presenting with a macrophage activating syndrome [MAS]. We also present a review of the literature that supports the diagnosis of SoJIA presenting with MAS

Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases

The aim of this research was to explore whether IL-18 can be a serological marker for the diagnosis of systemic-onset juvenile idiopathic arthritis (sJIA). A total of 23 sJIA patients (13 males, median age 8.2), 20 acute lymphoblastic leukemia (ALL) patients, 18 patients with severe infections (SIF), 26 Kawasaki disease (KD) patients, 18 juvenile idiopathic arthritis (JIA) patients, and 25 healthy control patients were selected for this study. Enzyme-linked immunosorbent assays (ELISAs) were used to determine the serum concentrations of the S100A8, S100A9, and IL-6 proteins. The serum IL-18 levels were detected by a cytometric bead array (CBA). The serum IL-6 concentrations in various disease groups were significantly higher than that in the healthy control group. The IL-6 concentrations exhibited no significant difference between disease groups. The S100A8 level in the sJIA group was significantly higher than those of the ALL, JIA, and healthy control groups but showed no significant difference compared to the SIF and KD groups. The S100A9 serum concentration in the sJIA group was significantly higher than those in the ALL and healthy control groups and exhibited no significant difference from the SIF, KD, and JIA groups. The IL-18 level of the sJIA group was significantly higher than that of the other febrile disease groups. The IL-18 serum concentration may be used as a biological serum marker to distinguish sJIA from other febrile diseases.

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.

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. .

Dois pares de irmãos com artrite idiopática juvenil (AIJ): relato de casos / Two pairs of brothers with juvenile idiopathic arthritis (JIA): case reports

Relato de casos de ocorrência de Artrite Idiopática Juvenil (AIJ) em dois pares de irmãos acompanhados no serviço de reumatologia pediátrica da Universidade Federal da Bahia. O envolvimento genético na patogênese da AIJ está claro e o risco de recorrência entre irmãos corrobora esta contribuição. Um importante marco dessa descoberta envolve a confirmação da contribuição dos polimorfismos do complexo principal de histocompatibilidade (MHC) na susceptibilidade ao desenvolvimento da AIJ. Apesar de muitos progressos, os inúmeros estudos existentes ainda não são capazes de explicar diversos mecanismos implícitos na patogênese da AIJ.

This is a case report of juvenile idiopathic arthritis in two pairs of brothers followed in the Department of Pediatric Rheumatology, Universidade Federal da Bahia. Genetic involvement in juvenile idiopathic arthritis pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex polymorphism contribution to juvenile idiopathic arthritis development susceptibility. Despite many advances, the numerousavailable studies cannot explain several implicit mechanisms in juvenile idiopathic arthritispathogenesis yet.

Guía clínica GES de artritis idiopática juvenil 2014 / 2014 GES clinical guideline of juvenile idiopathic arthritis

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

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

Phenotype Difference between Familial and Sporadic Ankylosing Spondylitis in Korean Patients

Clustered occurrences of ankylosing spondylitis (AS) in family have been noticed. We evaluated patients with AS confirmed by the modified New York criteria for familial history of AS (one or more first to third degree relatives). The clinical characteristics and the recurrence risks (number of AS patients/number of familial members) of the familial AS compared to sporadic AS were investigated. Out of a total of 204 AS patients, 38 patients (18.6%) reported that they had a familial history of AS. The recurrence risks in the familial AS patients for first, second and third degree family members were 14.5%, 5.2%, and 4.4% respectively. Erythrocyte sedimentation rate (ESR) (22.6+/-22.2 vs 35.4+/-34.4, P=0.029) and C-reactive protein (CRP) (1.24+/-1.7 vs 2.43+/-3.3, P=0.003) at diagnosis, body mass index (21.9+/-2.7 vs 23.7+/-3.3, P=0.002) and frequency of oligoarthritis (13.2% vs 33.7%, P=0.021) were significantly lower in the familial form. The presence of HLA-B27 (97.4% vs 83.1%, P=0.044) was significantly higher in familial AS. In conclusion, Korean familial AS patients show a lower frequency of oligoarthritis, lower BMI, lower ESR and CRP at diagnosis and higher presence of HLA-B27.

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

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

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

Patología inflamatoria de columna en niños y adolescentes / Inflammatory spine pathology in children and adolescents

Chronic inflammatory axial pain is an uncommon pediatric syndrome, brings a number of diseases affecting the axial skeleton. It is characterized by unknown etiology, with recognizing genetic susceptibility factors. The medical clinician should be performed to establish the diagnosis, making accurate therapy for long-term success and working to get a good quality of life. Current classifications established for children and young patients forms are limited by the pediatric medical short follow-up age. Two international classifications (a) International League of Associations for Rheumatology and (b) Classification of juvenile spondyloarthropathies Spondylarthropathy European group Study Group to achieve approximate diagnosis for pediatric rheumatology forms. The adult rheumatologist usually who will establish the definitive diagnosis and prognosis. The chronic inflammatory axial pain needs an unification of classification criteria for children and adults in order to facilitate the scientific communication and medical transition.

El dolor axial inflamatorio crónico es una entidad infrecuente en Pediatría, y agrupa una serie de patologías que afectan el esqueleto axial. este grupo de enfermedades son de etiología aún desconocida, reconociendo factores de susceptibilidad genética en ellas. Su importancia está en el enfoque que el clínico debe realizar para establecer el diagnóstico, realizar una terapia precoz para obtener buenos resultados a largo plazo y procurar que el paciente obtenga una buena calidad de vida. Las clasificaciones actuales establecidas para las formas infantojuveniles se ven limitadas por lo breve del periodo de seguimiento etario, además que se hace necesario aplicar dos clasificaciones internacionales (a) International League of Associations for Rheumatology y (b) Clasificación de Espondiloartropatías Juveniles del European Spondyloarthropathy Study Group para lograr el diagnóstico aproximado. Es necesario considerar que en muchos casos será el reumatólogo de adultos quien establecerá el diagnóstico y pronóstico definitivo. Se reconoce que este grupo de patología inflamatoria crónica requiere unificación de criterios de clasificación en niños y adultos para facilitar la comunicación científica y de transición.

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.