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.

Doença periodontal e artrite idiopática juvenil: uma inter-relação bidirecional / Periodontal disease and juvenile idiopathic arthritis: a bidirectional inter-relationship

Parece haver similaridades entre a patogenia de doenças reumatológicas (artrite reumatoide e artrite idiopática juvenil) e periodontite. Alguns estudos têm sido conduzidos com o objetivo de elucidar os mecanismos que explicam a inter-relação entre essas condições. A artrite reumatoide parece aumentar a suscetibilidade à doença periodontal destrutiva em adultos e em pacientes com artrite idiopática juvenil. No entanto, ainda são pouco conhecidas as vias de associação entre essas condições crônicas infl amatórias (periodontite e artrite). Desta forma, o objetivo deste trabalho foi promover uma revisão da literatura sobre a inter-relação artrite idiopática infantil e doença periodontal.

Similarities between rheumatologic diseases (rheumatoid arthritis and juvenile idiopathic arthritis) and periodontitis pathogenesis are discussed. Some studies have been conducted to elucidate the mechanisms that explain the relationship between these conditions. Rheumatoid arthritis appears to increase susceptibility to destructive periodontal disease in adults and in patients with juvenile idiopathic arthritis. However, the pathway of association between these chronic infl ammatory conditions are barely known. Thus, the aim of this study was to review the literature concerning the relationship between juvenile idiopathic arthritis and periodontitis.

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...

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.

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.

Anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis.

Objective. Prevalence and clinical significance of anti-cyclic citrullinated peptide (CCP) antibodies in Indian patients with juvenile idiopathic arthritis (JIA). Methods. Anti-CCP antibodies were determined by enzyme-linked immunosorbent assay (ELISA) in 78 patients with JIA which included all 3 major subtypes of the disease: pauciarticular, polyarticular afld systemic onset. Values above 5 relative units were taken as positive. Associations between antiCCP antibodies and clinical and laboratory and radiological parameters were determined. Results. Anti-CCP antibodies were positive in only 2 of 34 (5.9%) patients with pauciarticular JIA and 3 of 17 (17.6%) of systemic,.pnset JIA, whereas it was positive in 13 of 27 (48.1%) of polyarticular JIA patients (p < 0.001). Furthermore, it was seen that among patients with polyarticular JIA, RF-lgM positive patients had higher rate of anti-CCP antibody positivity with 7 of 8 (87.5%) patients having positive anti-CCP antibody (p<0.001). Similarly, patients with erosions (11/19; p<0.001) and deformities (5/-10; p<0.001) were found to have significant association with anti-CCP antibody positivity. Conclusion. Anti-CCP antibodies could be detected more frequently in the sera of JIA patients with severe manifestations like- erosions and deformity. It was also more significantly associated with seropositive polyarticular JIA than other types. It can be presumed from these results that anti-CCP antibodies can be used as a marker to predict severe course of JIA at the onset to guide optimal aggressive therapy.

Artritis idiopática juvenil / Juvenile idiopathic arthritis

La Artritis Idiopática Juvenil (AIJ), es un término que describe a un grupo heterogéneo de artritis inflamatoria crónica, que ocurre en personas menores de 16 años de edad, tiene una duración de 6 semanas o más, y donde otras patologías han sido excluidas. El propósito de esta revisión es presentar los cambios en la clasificación y diagnóstico, y mencionar algunos aspectos en el tratamiento de la Artritis Idiopática Juvenil, a fin de lograr un mayor conocimiento de esta patología, en cuyo seguimiento intervienen de manera multidisciplinaria el pediatra, el reumatólogo infantil, el fisiatra, el traumatólogo, el psicólogo y los especialistas de sistemas afectados.

Juvenile idiopathic arthritis (JIA) is a term that describes a heterogeneous group of chronic inflammatory arthritic conditions occurring in persons under age 16 and which last 6 weeks or more, when other diseases have been excluded. The purpose of this review is to report on changes that have been made in classification and diagnosis of the condition, and to mention some aspects of its treatment to promote better understanding of these conditions, in which roles exist for practitioners from a number of disciplines, including pediatricians, pediatric rheumatologists, physiatrists, traumatologists, psychologists, and others from specialties related to specific affected systems.

Artritis idiopática juvenil: parte 1: diagnóstico, patogenia y manifestaciones clínicas / Juvenil idiopathic arthritis: part 1: diagnosis, pathogenesis and clinical manifestations

La artritis idiopática juvenil no constituye una entidad “única” sino un grupo heterogéneo de enfermedades o trastornos inflamatorios. Esta nueva denominación abarca diferentes categorías de enfermedad, cada una de ellas con distintas formas de presentación, signos y síntomas clínicos, y pronóstico. La causa de esta entidad es aún desconocida, pero factores ambientales y genéticos intervienen en su patogenia. Es la más común de las enfermedades reumáticas en la infancia y causa importante de discapacidad a corto y largo plazos. Revisaremos aquí las manifestaciones clínicas, la nueva clasificación, el abordaje diagnóstico y los diagnósticos diferenciales.

Juvenile idiopathic arthritis is not a single disease and constitutes an heterogeneous group of illnesses or inflammatory disorders. This new nomenclature encompasses different disease categories, each of which has different presentation, clinical signs, symptoms, and outcome. The cause of the disease is still unknown but both environmental and genetic factors seem to be related to its pathogenesis. Is the most common chronic rheumatic disease in children and an important cause of short-term and long-term disability.In this article, clinical manifestation, new classification and approach to diagnosis are reviewed.

Guía de tratamiento de la artritis idiopática juvenil / Treatment guide of juvenile idiopathic arthritis

Las artritis inflamatorias del niño constituyen un grupo heterogéneo de enfermedades de presentaciones clínicasdiversas y distintas bases genéticas. Esto ha hecho necesario desarrollar protocolos para el mejor manejo de estos cuadros. En este artículo el Grupo Pediátrico de la Sociedad Chilena de Reumatología ha propuesto una Guía clínica de tratamiento de la Artritis Idiopática Juvenil según los actualesCriterios de Clasificación de ILAR (International League of Associatons for Rheumatology), Edmonton 2001.

Inflammatory arthritis in children is a heterogeneous disease group with several clinical signs and different genetic background. This has brought about the need to develop clinical trials to improve disease management. In this article, the Pediatric Group of the Chilean Rheumatology Society has proposed a Clinical Guide for the medical treatment of Juvenile Idiopathic Arthritis, based on the latest Classification Criteria of the International League of Associations for Rheumatology, ILAR, Edmonton 2001.

Durban 1997 ¿una propuesta viable para la clasificación de artritis juvenil? / Durban 1997: a viable proposal for juvenil arthritis classification?

Las Artritis Juveniles son un grupo heterogéneo de padecimientos que a través del tiempo han venido recibiendo diferentes nombres y se han tratado de agrupar de diversas maneras; sin embargo, no se ha logrado un consenso internacional para lograr este fin y es conocida la intención de establecer una clasificación que permita el estudio y seguimiento de estas artritis. En Europa y Estados Unidos de América se han propuesto diferentes clasificaciones en las que han participado algunas organizaciones como el Colegio Americano de Reumatología, la Organización Mundial de la Salud y la Liga Europea contra el Reumatismo. Preocupados por lograr una clasificación que permitiera el estudio de estos padecimientos, la Liga Internacional de Asociaciones de Reumatología formó una fuerza de trabajo para proponer una nueva clasificación; la última reunión de este grupo en Durban, Sudáfrica en 1997 generó una última propuesta de clasificación de la que se aquí se hace una revisión y comparación con clasificaciones previas.

Clinico-immunological profile of juvenile rheumatoid arthritis at Chandigarh.

OBJECTIVE: To study the clinical and immunological profile of children with juvenile rheumatoid arthritis (JRA). DESIGN: Retrospective hospital based study. SETTING: Tertiary level center of North India. SUBJECTS:74 patients attending the Pediatric Rheumatology and Immunology Clinic over last 5 years. RESULTS: The patients were aged between 9 months to 12 years with male female ratio of 1.8:1. Eleven (14.9%) patients had systemic onset JRA, 28 (37.8%) had polyarticular onset type and 35(47.3%) had pauciarticular onset type JRA. Uveitis was present only in one patient and rheumatoid nodules were present in 4(5.4%) patients. Rheumatoid factor was positive in 2(2.7%) and antinuclear antibody was present in one patient only. HLA-B27 was positive in 4 children. Two patients developed amyloidosis. CONCLUSION:The clinico-immunological profile of JRA at Chandigarh appears to be some what different from that reported from other centers in India.

Juvenile rheumatoid arthritis--Madras experience.

In a prospective study of 1,053 consecutive children who attended the Rheumatic Care Centre, Government General Hospital, Madras from 1991 to 1995, 331 children fulfilled the criteria proposed by the American Rheumatism Association as modified by Cassidy et al for the diagnosis of Juvenile Rheumatoid Arthritis. These children were thoroughly examined and investigated and classified into 3 onset types which was then sub-classified into early entry and late entry groups based on the duration of illness. Other arthritic conditions were excluded. There were 44 cases belonging to Systemic onset, 171 belonging to polyarticular onset and 116 belonging to oligoarticular onset type. In the systemic onset type 44/44 patients had fever, 40/44 had lymphadenopathy and 19/44 had skin rash; wrists and knees 31/44 were the most commonly involved joints; neck involvement was present in 13/44 of the cases; ANA was positive in 5/44 cases and anaemia was seen in 24/44 cases. In polyarticular onset type wrists 119/171, knees 143/171, hip joints 105/171 and ankles 113/171 were commonly involved; in the RF +ve subtype 3/23 had subcutaneous nodules and 7/23 were positive for ANA; in the Rf -ve subtype 59/148 were positive for ANA. In the oligoarticular subtype-1 ANA was positive in all cases but iridocyclitis was not seen in any case. In oligoarticular subtype-2 HLA B27 was positive in 13/26 cases while Sacroilitis was seen in 16/26 cases. In oligoarticular type-3 HLA B27 was negative.

Juvenile rheumatoid arthritis in India--rarity of antinuclear antibody and uveitis.

Juvenile chronic arthritis is a heterogeneous disease, having different subtypes. Among our 89 patients with juvenile chronic arthritis, we did not find even one patient with early onset pauciarticular disease with uveitis and antinuclear antibody positivity. Further, the prevalence of anti-nuclear antibodies and anti-histone antibodies was very low whereas the prevalence of rheumatoid factor was similar to that reported from Western countries. Thus, the spectrum of juvenile chronic arthritis in India differs from that seen in the west. Larger population based studies of the disease are thus needed.

Clinico-immunological profile in juvenile rheumatoid arthritis--an Indian experience.

From a Pediatric Rheumatology Clinic 361 children diagnosed as juvenile rheumatoid arthritis (JRA) according to American Rheumatism Association-JRA criteria were studied retrospectively for their clinico-immunological profile. The mean age of onset in systemic, pauciarticular and polyarticular onset, JRA subtypes were 5.2, 6.8 and 7.2 years respectively. There was male preponderance in systemic and pauciarticular JRA. In seropositive polyarticular JRA, girls outnumbered boys. The frequency of occurrence of systemic, pauciarticular and polyarticular disease was 87 (24%), 108 (30%) and 166 (46%) respectively. The systemic onset disease was dominated by extra-articular manifestations in terms of fever (100%), rash (57%), hepatomegaly (51%) and lymphadenopathy (25%). The pauci- and polyarticular illnesses were commonly dominated by joint involvement, morning stiffness, and in few patients, by extra-articular manifestations also. The joints were involved symmetrically. Most commonly involved joints in order of decreasing frequency were knee, ankle, wrist and elbow in all the subtypes. Anemia and leucocytosis were observed in majority with higher frequency in systemic onset JRA. The rheumatoid factor (RF) was present in 15% of polyarticular JRA. RF was also present in 7 and 9% of patients with pauciarticular and systemic subtypes respectively. The antinuclear antibody was positive in only 3 out of 66 patients in whom the test was carried out. The demographic profile and trends in clinical features were similar to the studies reported on caucasian population with difference in the actual frequency of various clinical features.

Juvenile rheumatoid arthritis--clinical manifestations.

Chronic inflammatory arthritis in childhood could be due to an obvious cause (e.g. sepsis, rheumatic fever, systemic lupus erythematosus etc.), or it could be idiopathic. After excluding those with obvious cause there still remains a large group of chronic inflammatory arthritis in childhood. This category has been variously called 'juvenile rheumatoid arthritis', 'juvenile arthritis', 'juvenile chronic arthritis', and more recently, 'idiopathic arthritis of childhood', The present article reviews the various classification criteria used for defining this group of disorders with emphasis on the common features as well as the major differences between these criteria. The major classes within this group with their characteristic clinical and laboratory features are also discussed.