Efectividad del etanercept en el tratamiento de la artritis idiopática juvenil / Efficacy of etanercept in the treatment of juvenile idiopathic arthritis

Objetivos: Los objetivos de este estudio son evaluar la efectividad del etanercept en el tratamiento de la artritis idiopática juvenil (AIJ) a los 6 meses de tratamiento; valorar la contribución de diferentes variables clínico demográficas en la respuesta, y valorar la seguridad y la tolerancia del fármaco a los 12 meses de tratamiento. Pacientes y métodos: Estudio retrospectivo de pacientes con AIJ que iniciaron tratamiento con etanercept entre enero de 2000 y junio de 2007. Se recogieron variables clínico demográficas y variables analíticas antes de iniciar el tratamiento y después de 3 y 6 meses. La mejoría se evaluó mediante el índice ACRped (criterio de mejoría pediátrico del ACR [American College of Rheumatology `Colegio Americano de Reumatología’]). Resultados: Se estudiaron 71 pacientes. A los 6 meses del tratamiento el 77,5% de los pacientes alcanzó el ACRped30, el 70,4% de los pacientes alcanzó el ACRped50 y el 54,9% de los pacientes alcanzó el ACRped70. La mejoría según el ACRped 30 fue del 100% en pacientes con artritis y entesitis, del 91,7% en pacientes con oligoartritis y del 66,7% en pacientes con poliartritis. Aunque 19 de los niños con AIJ sistémica (63%) alcanzaron una mejoría ACRped30, la fiebre no se controló en ninguno de los 12 pacientes que la presentaban y todos (menos 3 pacientes) precisaron aumento de corticoides sistémicos o infiltraciones articulares con esteroides. El etanercept se suspendió en 12 pacientes, en 6 durante el primer semestre y en 6 durante el segundo semestre. En 7 pacientes el tratamiento se interrumpió por ineficacia o recaída. El 80,3% de los pacientes continuaba el tratamiento a los 12 meses. Conclusiones: El etanercept es un tratamiento efectivo y bien tolerado en niños con AIJ, aunque es de escasa utilidad en pacientes con formas sistémicas (AU)

Objectives: 1) To assess the efficacy of etanercept in the treatment of children with juvenile idiopathic arthritis (JIA) after 6 months of therapy, 2) to analyse the contribution of several variables in the clinical response, and 3) to evaluate the safety and tolerability of the drug after 12 months of treatment. Patients and methods: Retrospective chart review of JIA patients started on etanercept from January 2000 to June 2007. The same clinical and laboratory variables were considered at the beginning of therapy and 3 and 6 months afterwards. Clinical improvement was defined according to the American College of Rheumatology pediatric criteria for improvement (ACRped). Results: Seventy one patients were studied. The ACRpedi30 response was achieved by 77.5% of the children, the ACRped50 by 70.4% and the ACRped70 by 54.9%. All children (100%) with enthesitis related arthritis, 91.7% of those with oligoarthritis and 66.7% with polyarthritis responded according to ACRped30. An ACRped30 response was achieved by 63% of children with systemic JIA (n=19), although fever was not controlled in any of the 12 children who presented it, and all but three required an increase in the steroid dose (oral and/or intra-articular). Etanercept was discontinued in 12 patients, 6 during the first six months and six between 7 and 12 months of therapy. In 7 of them because of inefficacy or flare-up. At the 12 month follow-up 80.3% of the patients continued taking etanercept. Conclusion: Etanercept is effective for the treatment of JIA and is well tolerated by children. Its clinical usefulness is very much reduced in children with systemic JIA (AU)

[Efficacy of etanercept in the treatment of juvenile idiopathic arthritis]. / Efectividad del etanercept en el tratamiento de la artritis idiopática juvenil.

OBJECTIVES: 1) To assess the efficacy of etanercept in the treatment of children with juvenile idiopathic arthritis (JIA) after 6 months of therapy, 2) to analyse the contribution of several variables in the clinical response, and 3) to evaluate the safety and tolerability of the drug after 12 months of treatment. PATIENTS AND METHODS: Retrospective chart review of JIA patients started on etanercept from January 2000 to June 2007. The same clinical and laboratory variables were considered at the beginning of therapy and 3 and 6 months afterwards. Clinical improvement was defined according to the American College of Rheumatology pediatric criteria for improvement (ACRped). RESULTS: Seventy one patients were studied. The ACRpedi30 response was achieved by 77.5% of the children, the ACRped50 by 70.4% and the ACRped70 by 54.9%. All children (100%) with enthesitis related arthritis, 91.7% of those with oligoarthritis and 66.7% with polyarthritis responded according to ACRped30. An ACRped30 response was achieved by 63% of children with systemic JIA (n=19), although fever was not controlled in any of the 12 children who presented it, and all but three required an increase in the steroid dose (oral and/or intra-articular). Etanercept was discontinued in 12 patients, 6 during the first six months and six between 7 and 12 months of therapy. In 7 of them because of inefficacy or flare-up. At the 12 month follow-up 80.3% of the patients continued taking etanercept. CONCLUSION: Etanercept is effective for the treatment of JIA and is well tolerated by children. Its clinical usefulness is very much reduced in children with systemic JIA.

[Thyroid disorders and childhood rheumatic diseases]. / Alteraciones tiroideas y enfermedades reumáticas infantiles.

INTRODUCTION: The relationship between thyroid dysfunction and autoimmune diseases has mainly been described in adults. The aim of this study was to analyse the prevalence and characteristics of thyroid abnormalities in children with rheumatic diseases. PATIENTS AND METHOD: One hundred and forty-five patients (109 girls and 36 boys) from a rheumatology paediatric unit were studied for two years. The diagnoses were: juvenile idiopathic arthritis (JIA) (n=115), lupus (n=17), juvenile dermatomyositis (n=5), scleroderma (n=4), and one case each of the following: mixed connective mixed disease, CINCA syndrome (chronic infantile neurological, cutaneous and articular), TRAPS (tumour necrosis factor receptor-associated periodic syndrome), and familial mediterranean fever. T4 and TSH levels were carried out, and if these showed abnormalities, antithyroid antibodies (ATA) were determined. RESULTS: Six girls aged between 2 and 17 years old had thyroid abnormalities. Three had JIA and three had lupus. Five were diagnosed with autoimmune hypothyroidism, with high ATA levels, and there was one case of hyperthyroidism. All of the patients with thyroid dysfunction had positive antinuclear antibodies (ANA), compared to 34.5% of the rest of the patients (p=0.003). CONCLUSIONS: The prevalence of thyroid abnormalities in children with rheumatic disease was 4.14% to 7.9% in JIA patients with positive ANA, and up to 17.6% with lupus. The majority of patients were asymptomatic. Thyroid hormones should be determined when rheumatic disease is diagnosed and periodically afterwards.

Alteraciones tiroideas y enfermedades reumáticas infantiles / Thyroid Disorders and Childhood Rheumatic Diseases

Introducción: la asociación de trastornos tiroideos y enfermedades autoinmunitarias ha sido descrita en adultos y, en menos ocasiones, en niños. El objetivo de este trabajo es analizar la prevalencia y las características de las alteraciones tiroideas en niños con enfermedades reumáticas. Pacientes y método: se estudió a 145 pacientes (109 mujeres y 36 varones) atendidos en una unidad de reumatología pediátrica durante 2 años. Los diagnósticos fueron: artritis idiopática juvenil (AIJ) (n=115), lupus (n=17), dermatomiositis juvenil (n=5), esclerodermia (n=4) y 1 caso de cada uno de los siguientes: enfermedad mixta del tejido conectivo, síndrome CINCA (chronic infantile neurologic cutaneous and articular), TRAPS (tumor necrosis factor receptor-associated periodic syndrome) y fiebre mediterránea familiar. En todos se determinaron las concentraciones de T4 y TSH, y si estaban alterados, se determinaban los anticuerpos antitiroideos (ATA). Resultados: 6 niñas tuvieron alteración tiroidea, con edades de 2 a 17 años; 3 tenían AIJ y 3, lupus; 5 fueron diagnosticadas de hipotiroidismo autoinmunitario, con ATA elevados, y 1 caso, de hipertiroidismo. El 100% de los pacientes con alteración tiroidea tenían anticuerpos antinucleares (ANA) positivos, frente al 37,4% de los restantes (p=0,003). Conclusiones: La prevalencia de alteraciones tiroideas en niños con enfermedad reumática fue del 4,14%, y aumentó al 7,9% en AIJ con ANA positivos y al 17,6% en lupus. La mayoría estaban asintomáticos. La determinación de hormonas tiroideas debería realizarse al diagnóstico de enfermedad reumática y de forma periódica después (AU)

Introduction: The relationship between thyroid dysfunction and autoimmune diseases has mainly been described in adults. The aim of this study was to analyse the prevalence and characteristics of thyroid abnormalities in children with rheumatic diseases. Patients and method: One hundred and forty-five patients (109 girls and 36 boys) from a rheumatology paediatric unit were studied for two years. The diagnoses were: juvenile idiopathic arthritis (JIA) (n=115), lupus (n=17), juvenile dermatomyositis (n=5), scleroderma (n=4), and one case each of the following: mixed connective mixed disease, CINCA syndrome (chronic infantile neurological, cutaneous and articular), TRAPS (tumour necrosis factor receptor-associated periodic syndrome), and familial mediterranean fever. T4 and TSH levels were carried out, and if these showed abnormalities, antithyroid antibodies (ATA) were determined. Results: Six girls aged between 2 and 17 years old had thyroid abnormalities. Three had JIA and three had lupus. Five were diagnosed with autoimmune hypothyroidism, with high ATA levels, and there was one case of hyperthyroidism. All of the patients with thyroid dysfunction had positive antinuclear antibodies (ANA), compared to 34.5% of the rest of the patients (p=0.003). Conclusions: The prevalence of thyroid abnormalities in children with rheumatic disease was 4.14% to 7.9% in JIA patients with positive ANA, and up to 17.6% with lupus. The majority of patients were asymptomatic. Thyroid hormones should be determined when rheumatic disease is diagnosed and periodically afterwards (AU)

[Macrophage activation syndrome and juvenile idiopathic arthritis. A multicenter study]. / Síndrome de activación macrofágica y artritis idiopática juvenil. Resultados de un estudio multicéntrico.

INTRODUCTION: Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (sJIA). OBJECTIVE: To describe the clinical characteristics and outcome of patients diagnosed with MAS in Spanish pediatric rheumatology units. PATIENTS AND METHOD: A protocol for data collection was designed and distributed to pediatricians and rheumatologists attending children with rheumatic diseases. RESULTS: Information was available from 31 patients (16 boys and 15 girls) who had 37 MAS episodes. Twenty-seven children had only one episode, three had two episodes and one had four episodes. The interval between episodes ranged from 1 to 33 months. The median age was 5.9 years (range 1-23). MAS was the initial manifestation of sJIA in nine patients. The most frequent symptom was fever (97%), followed by skin rash (49%), central nervous system dysfunction (41%), and gastrointestinal abnormalities (15%). Abnormal laboratory findings included thrombopenia (78%) and elevated levels of hepatic enzymes (70%). Hemophagocytosis was confirmed in 16 of 30 bone marrow samples evaluated, 15 with cyclosporine A and six with etoposide. All episodes but one were treated with steroids. One patient received a liver transplant before diagnosis. The mortality rate was 6.5% (2/31). CONCLUSION: MAS is a severe, potentially lethal, complication of sJIA. The clinical and laboratory abnormalities characteristic of sJIA complicate its diagnosis. The earliest and most frequent findings were decreased in platelet count and elevation of hepatic enzymes. A high degree of suspicion as well as early diagnosis and prompt treatment are essential in this disease.

Síndrome de activación macrofágica y artritis idiopática juvenil. Resultados de un estudio multicéntrico / Macrophage Activation Syndrome and Juvenile Idiopathic Arthritis. A multicenter study

Introducción: El síndrome de activación macrofágica (SAM) es una complicación grave de la artritis idiopática juvenil con forma de comienzo sistémico (AIJ-S). Objetivo Describir las características clínicas y la evolución de los pacientes con SAM diagnosticados en las unidades de reumatología pediátrica en España. Pacientes y método: Se elaboró un protocolo de recogida de datos que se distribuyó a pediatras y reumatólogos que atienden niños con enfermedades reumáticas. Resultados: Se recogieron datos de 31 pacientes (16 varones y 15 mujeres) que desarrollaron 37 episodios de SAM. Un total de 27 tuvieron un solo episodio; en 3 casos hubo dos episodios y 1 tuvo cuatro episodios, con intervalos entre ellos de 1 a 33 meses. La media de edad en el primer episodio fue 5,9 años (rango 1-23). El SAM fue la primera manifestación de AIJ-S en 9 casos. El síntoma más frecuente fue la fiebre (97 %), seguido de exantema (49 %), alteraciones neurológicas (41 %) y digestivas (15 %). Los datos analíticos más frecuentes fueron trombocitopenia (78 %) y aumento de las enzimas hepáticas (70 %). En 16 de 30 médulas óseas examinadas se objetivó hemofagocitosis. Todos los episodios menos uno fueron tratados con corticoides. Ciclosporina A fue utilizada en 15 episodios y etopósido, en seis. En un paciente se realizó un trasplante hepático previo al diagnóstico de SAM. El nivel de mortalidad fue del 6,5 % (2/31). Conclusiones: El SAM es una complicación grave, potencialmente fatal, de la AIJ-S. Las alteraciones características de la enfermedad de base dificultan el diagnóstico. La disminución en el recuento de plaquetas y la elevación de las enzimas hepáticas fueron los hallazgos más frecuentes y precoces. Un alto grado de sospecha, un diagnóstico y un tratamiento precoces son esenciales para la resolución del cuadro (AU)

Introduction: Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (sJIA). Objective: To describe the clinical characteristics and outcome of patients diagnosed with MAS in Spanish pediatric rheumatology units. Patients and method: A protocol for data collection was designed and distributed to pediatricians and rheumatologists attending children with rheumatic diseases. Results: Information was available from 31 patients (16 boys and 15 girls) who had 37 MAS episodes. Twenty-seven children had only one episode, three had two episodes and one had four episodes. The interval between episodes ranged from 1 to 33 months. The median age was 5.9 years (range 1-23). MAS was the initial manifestation of sJIA in nine patients. The most frequent symptom was fever (97 %), followed by skin rash (49 %), central nervous system dysfunction (41 %), and gastrointestinal abnormalities (15 %). Abnormal laboratory findings included thrombopenia (78 %) and elevated levels of hepatic enzymes (70 %). Hemophagocytosis was confirmed in 16 of 30 bone marrow samples evaluated, 15 with cyclosporine A and six with etoposide. All episodes but one were treated with steroids. One patient received a liver transplant before diagnosis. The mortality rate was 6.5 % (2/31). Conclusion: MAS is a severe, potentially lethal, complication of sJIA. The clinical and laboratory abnormalities characteristic of sJIA complicate its diagnosis. The earliest and most frequent findings were decreased in platelet count and elevation of hepatic enzymes. A high degree of suspicion as well as early diagnosis and prompt treatment are essential in this disease (AU)

[Evaluation of a clinical pathway for septic arthritis]. / Evaluación de una vía clínica de artritis séptica.

INTRODUCTION: Clinical pathways are tools that coordinate clinical work, reducing interclinican variability and improving patient care and management. The use of clinical pathways in septic arthritis is appropriate, as this disease has a predictable course and there is considerable variation in its management. The aim of this study was to evaluate a septic arthritis clinical pathway 2 years after its introduction and to describe the characteristics of the patients included. MATERIAL AND METHODS: Clinical pathway documents: pathway matrix sheet, variance form, parent information sheet, satisfaction survey and evaluation indicators sheet. RESULTS: Thirty-five patients were included, seven with a definitive diagnosis of septic arthritis and 28 with probable septic arthritis. No differences were found between the two groups, with good outcomes in both. Laboratory analyses were performed at admission in all patients, at discharge in 51 %, and at the end of treatment in 97 %. The indicators that best met the standard were clinical pathway coverage, performance of arthrocentesis/arthrotomy, and satisfaction with dealings with staff and the information received. The indicators furthest from the standard were admission shorter than 7 days (77 % vs > 95 %) and obtaining articular fluid prior to antibiotic therapy (76 % vs > 90 %). CONCLUSIONS: The clinical pathway is useful for standardizing the process of septic arthritis diagnosis and treatment. With adequate clinical support, application of this pathway allows decisions to be made on hospital discharge following the clinical criteria of improvement without worsening prognosis. Our immediate challenges are to reduce inpatient stay and to obtain synovial fluid before starting antibiotic therapy.

Evaluación de una vía clínica de artritis séptica / Evaluation of a clinical pathway for septic arthritis

Introducción. Una vía clínica es una herramienta que coordina la labor asistencial, disminuyendo la variabilidad entre profesionales, al tiempo que mejora la atención a los pacientes y la gestión. La artritis séptica es candidata a ser abordada mediante una vía clínica porque su evolución es predecible y existe gran diversidad en el manejo. El objetivo del trabajo es evaluar una vía clínica tras 2 años de implantación y describir las características de los pacientes que fueron incluidos. Material y métodos. Documentación de la vía: matriz, hoja de variaciones, hoja informativa, encuesta de satisfacción e indicadores de evaluación. Resultados. Se incluyeron 35 pacientes, siete artritis sépticas seguras y 28 probables. No hubo diferencias entre los 2 grupos, siendo su evolución favorable. Se practicó analítica al ingreso a todos los pacientes, al alta hospitalaria al 51 % y al alta definitiva al 97 %. Los mejores indicadores de evaluación fueron la cobertura de la vía, la realización de artrocentesis/artrotomía y la satisfacción en el trato o información recibidos y los indicadores más alejados del estándar fueron la hospitalización menor de 7 días (77 % frente a > 95 %) y la obtención de líquido articular sin antibióticos (76 % frente a > 90 %). Conclusiones. La vía clínica resulta útil para estandarizar el proceso de diagnóstico y tratamiento de la artritis séptica. Su aplicación con un soporte clínico adecuado permite decidir el alta hospitalaria siguiendo los criterios clínicos de mejoría, sin que por ello empeore el pronóstico. Optimizar el tiempo de hospitalización y conseguir líquido articular antes de iniciar el tratamiento antibiótico son nuestros retos inmediatos

Introduction. Clinical pathways are tools that coordinate clinical work, reducing interclinican variability and improving patient care and management. The use of clinical pathways in septic arthritis is appropriate, as this disease has a predictable course and there is considerable variation in its management. The aim of this study was to evaluate a septic arthritis clinical pathway 2 years after its introduction and to describe the characteristics of the patients included. Material and methods. Clinical pathway documents: pathway matrix sheet, variance form, parent information sheet, satisfaction survey and evaluation indicators sheet. Results. Thirty-five patients were included, seven with a definitive diagnosis of septic arthritis and 28 with probable septic arthritis. No differences were found between the two groups, with good outcomes in both. Laboratory analyses were performed at admission in all patients, at discharge in 51 %, and at the end of treatment in 97 %. The indicators that best met the standard were clinical pathway coverage, performance of arthrocentesis/arthrotomy, and satisfaction with dealings with staff and the information received. The indicators furthest from the standard were admission shorter than 7 days (77 % vs > 95 %) and obtaining articular fluid prior to antibiotic therapy (76 % vs > 90 %). Conclusions. The clinical pathway is useful for standardizing the process of septic arthritis diagnosis and treatment. With adequate clinical support, application of this pathway allows decisions to be made on hospital discharge following the clinical criteria of improvement without worsening prognosis. Our immediate challenges are to reduce inpatient stay and to obtain synovial fluid before starting antibiotic therapy

[Henoch-Schönlein purpura. Study of factors associated with the development and course of the disease]. / Púrpura de Schönlein-Henoch. Estudio de factores asociados con el desarrollo y evolución de la enfermedad.

INTRODUCTION: Henoch-Schönlein purpura (HSP) is the most common form of pediatric vasculitis. The objective of this study was to determine the factors associated with the development and course of this disease. PATIENTS AND METHODS: A case-control study was performed. The case group included patients with HSP followed-up at the pediatric rheumatology and nephrology units of a tertiary university hospital over a 2-year period. The control group included children followed-up at the pediatric rheumatology unit for mechanical or non-inflammatory conditions. A medical history including data on infectious conditions and previous medication was taken. A throat culture was performed and antistreptolysin 0 levels were quantified. The seroprevalence of different viruses was investigated. Subsequently, the patients were prospectively followed-up and disease manifestations were compared with reported epidemiological factors. RESULTS: Seventy patients and 58 controls were studied. A history of a recent upper respiratory infection (URI) and antibiotic intake were independently associated with development of HSP. Palpable purpura was present in 100 % of the patients. Gastrointestinal manifestations were recorded in 63 %, articular in 50 %, and renal in 18.6 %. Arthralgias were more frequent in girls and purpura duration was longer when disease onset occurred in spring or summer. Other factors studied were not associated with disease development or with a worse clinical course. CONCLUSIONS: Factors associated with the development of HSP were a history of URI and antibiotic administration. Other epidemiological factors studied were not associated with either the development or the course of the disease.

Púrpura de Schönlein-Henoch. Estudio de factores asociados con el desarrollo y evolución de la enfermedad / Henoch-Schönlein purpura. Study of factors associated with the development and course of the disease

Introducción: La púrpura de Schönlein-Henoch (PSH) es la vasculitis más frecuente en la infancia. El objetivo fue investigar posibles factores asociados con el desarrollo y evolución de la enfermedad. Pacientes y métodos: Se realizó un estudio caso-control. Los casos fueron los pacientes con PSH vistos en Reumatología y Nefrología Pediátrica de un hospital terciario durante 2 años y los controles los atendidos en Reumatología Pediátrica por problemas mecánicos o funcionales. Se recogieron antecedentes infecciosos, ingesta de medicación previa y se investigó la seroprevalencia de diferentes virus. Se realizó frotis faríngeo y se cuantificó la cifra de antiestreptolisina O. Posteriormente los pacientes fueron seguidos de forma prospectiva y se compararon las diversas manifestaciones de la enfermedad con los factores epidemiológicos referidos. Resultados: El número de pacientes fue 70 y el de los controles 58. Sólo el antecedente de infección del tracto respiratorio superior (ITRS) y la ingesta de antibióticos se asociaron de forma independiente con el desarrollo de PSH. El 100 % de los pacientes presentó púrpura palpable. Las manifestaciones digestivas fueron las más frecuentes (63 %), seguidas de las articulares (50 %) y de las renales (18,6 %). Las artralgias fueron más frecuentes en las niñas y el tiempo de duración de la púrpura fue más prolongado cuando la enfermedad empezó en primavera o verano. Ninguno de los otros factores epidemiológicos estudiados se asoció con el desarrollo de la enfermedad ni con parámetros de peor evolución. Conclusiones: El antecedente de ITRS y la ingesta de antibióticos se asociaron con el desarrollo de PSH. Otros factores epidemiológicos estudiados no se encontraron asociados con el desarrollo ni la evolución de la enfermedad

Introduction: Henoch-Schönlein purpura (HSP) is the most common form of pediatric vasculitis. The objective of this study was to determine the factors associated with the development and course of this disease. Patients and methods: A case-control study was performed. The case group included patients with HSP followed-up at the pediatric rheumatology and nephrology units of a tertiary university hospital over a 2-year period. The control group included children followed-up at the pediatric rheumatology unit for mechanical or non-inflammatory conditions. A medical history including data on infectious conditions and previous medication was taken. A throat culture was performed and antistreptolysin 0 levels were quantified. The seroprevalence of different viruses was investigated. Subsequently, the patients were prospectively followed-up and disease manifestations were compared with reported epidemiological factors. Results: Seventy patients and 58 controls were studied. A history of a recent upper respiratory infection (URI) and antibiotic intake were independently associated with development of HSP. Palpable purpura was present in 100 % of the patients. Gastrointestinal manifestations were recorded in 63 %, articular in 50 %, and renal in 18.6 %. Arthralgias were more frequent in girls and purpura duration was longer when disease onset occurred in spring or summer. Other factors studied were not associated with disease development or with a worse clinical course. Conclusions: Factors associated with the development of HSP were a history of URI and antibiotic administration. Other epidemiological factors studied were not associated with either the development or the course of the disease

Fibrodisplasia osificante progresiva: aportación de 2 casos / Progressive ossifying fibrodysplasia: Report of two cases

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[Malignant disease presenting as rheumatic manifestations]. / Manifestaciones reumáticas como forma de comienzo de enfermedad maligna.

BACKGROUND: Musculoskeletal pain is a frequent complaint in pediatrics in both tertiary and primary care. Although musculoskeletal symptoms are not usually related to severe disease, they can represent the first manifestation of an occult malignancy. OBJECTIVE: To describe the clinical manifestations and complementary findings of the patients referred to a pediatric rheumatology unit with a final diagnosis of malignancy. MATERIAL AND METHODS: We performed a retrospective review of all the children referred to a pediatric rheumatology unit between 1992 and 2002 whose final diagnosis was cancer. RESULTS: Of 3,982 patients referred, 10 had a final diagnosis of neoplasia (0.25 %). Six children were diagnosed with acute lymphoblastic leukemia, two with Langerhans' cell histiocytosis, one with Ewing's sarcoma, and one with metastases from retinoblastoma. The most frequent symptoms were arthralgias, limp, or back pain. Laboratory studies revealed anemia and a significant increase in sedimentation rate and lactate dehydrogenase values. Imaging studies contributed significantly to the diagnosis. The diagnosis was confirmed by bone marrow aspirate in children with leukemia and by bone biopsy in those with bone tumors. CONCLUSIONS: Malignancies should be considered in the differential diagnosis of children with musculoskeletal pain, especially in the presence of organomegalies or abnormal laboratory or imaging studies.

Manifestaciones reumáticas como forma de comienzo de enfermedad maligna / Malignant disease presenting as rheumatic manifestations

Antecedentes Los dolores musculoesqueléticos son un motivo frecuente de consulta tanto en Atención Primaria como en Reumatología Pediátrica. En la mayoría de las ocasiones se trata de síntomas inespecíficos sin relación con enfermedades graves, sin embargo pueden presentarse como síntoma inicial de un proceso neoplásico. Objetivo Describir las manifestaciones clínicas y analíticas de los pacientes vistos en una Unidad de Reumatología Pediátrica cuyo diagnóstico final fue neoplasia. Material y métodos Estudio retrospectivo de los pacientes remitidos a una Unidad de Reumatología Pediátrica durante los años 19922002, y cuyo diagnóstico final fue de malignidad. Resultados De 3.982 pacientes atendidos, 10 tuvieron una enfermedad tumoral (0,25 por ciento). Los diagnósticos finales fueron leucemia linfoblástica aguda en 6 casos, histiocitosis de células de Langerhans en dos, sarcoma de Ewing en uno y metástasis de retinoblastoma en otro. Los síntomas más frecuentes fueron artralgias, cojera y dolor en la columna. Entre las alteraciones analíticas destacaron el aumento de la velocidad de sedimentación globular, la anemia, y un incremento de los niveles de la enzima lactato deshidrogenasa. Las pruebas de imagen contribuyeron de forma significativa al diagnóstico. La confirmación del mismo se realizó mediante medulograma en las leucemias y por biopsia ósea en los tumores sólidos. Conclusiones Las neoplasias deben considerarse en el diagnóstico diferencial de los dolores osteoarticulares, sobre todo si van acompañados de visceromegalia, alteraciones analíticas o radiológicas (AU)

[Growth in children with juvenile idiopathic arthritis]. / Crecimiento en niños con artritis idiopática juvenil.

INTRODUCTION: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease that often requires steroid therapy. Growth retardation can be a serious complication in some of these patients. OBJECTIVE: To study linear growth in patients with JIA and evaluate the factors involved in its disturbance. METHODS: We studied 91 patients with JIA with a follow-up of at least one year. A cross sectional study, a longitudinal retrospective study, and a longitudinal prospective study were performed. Height in the first consultation, in the cross sectional study, and one year previously was evaluated. Height velocity (HV) was calculated. Several parameters related to disease activity, corticosteroid therapy, nutritional and hormonal status, and bone mineral density (BMD) were analyzed. A correlation study and multivariate regression analysis were carried out. RESULTS: Height was < or = -2 SD in 14.3 % of the series and in 55.6 % of the systemic group. Variables independently associated with height were total corticosteroid dose, functional class, nutritional index, BMD, and age at onset of the disease. HV was < or = 2 SD in 25.3 % of the series and in 61 % of the systemic group. Variables independently associated with HV were corticosteroid dose and the number of swollen joints. CONCLUSIONS: Growth retardation in patients with JIA was associated with factors related to the disease, corticosteroid therapy, nutritional status, BMD, and earlier onset of the disease.

Artritis idiopática juvenil: tratamiento actual y perspectivas terapéuticas / Juvenile idiophatic arthritis: current treatment and therapeutic perspectives

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Crecimiento en niños con artritis idiopática juvenil / Growth in children with juvenile idiopathic arthritis

Introducción. La artritis idiopática juvenil es una enfermedad inflamatoria crónica que precisa con frecuencia corticoterapia. El retraso del crecimiento puede ser un problema importante en estos pacientes. Objetivo. Estudiar el crecimiento de los niños con artritis idiopática juvenil y analizar los factores implicados en las alteraciones de éste. Métodos. Se estudiaron 91 pacientes con artritis idiopática juvenil, seguidos durante al menos un año. Se realizó un estudio de corte transversal, uno longitudinal retrospectivo y otro prospectivo. Se valoró la talla en la primera consulta, en el estudio transversal y un año antes. Se calculó la velocidad de crecimiento. Se estudiaron parámetros relacionados con la actividad de la enfermedad, con la corticoterapia, con el estado nutritivo, con la situación hormonal y la densidad mineral ósea (DMO). Se realizó un estudio de correlación y un análisis de regresión multivariante. Resultados. La talla fue inferior o igual a -2 desviaciones estándar (DE) en el 14,3 por ciento del total de los pacientes y en el 55,6 por ciento de las formas sistémicas. Los factores asociados de forma independiente con la talla fueron la dosis total de corticoides recibida, la clase funcional, el índice nutricional, la DMO y la edad de comienzo de la enfermedad. La velocidad de crecimiento fue inferior a lo normal en el 25,3 por ciento del total y en el 61 por ciento del grupo sistémico y los factores asociados de forma independiente con ella fueron la dosis de corticoide y el número de articulaciones inflamadas. Conclusiones. El retraso en el crecimiento en pacientes con artritis idiopática juvenil se asoció a factores relacionados con la enfermedad, con la corticoterapia, con el estado nutritivo, con la DMO y con el comienzo precoz de la enfermedad (AU)

[Uveitis and juvenile idiopathic arthritis]. / Uveítis y artritis idiopática juvenil.

OBJECTIVE: Anterior uveitis is one of the most important extra-articular manifestations of juvenile idiopathic arthritis (JIA). The objective was to analyze the frequency of uveitis in patients with JIA and to describe its clinical and evolutive characteristics. PATIENTS AND METHOD: Among the 234 children diagnosed with JIA in our hospital, those presenting uveitis were studied. RESULTS: Seventeen children, 16 girls and 1 boy, presented uveitis in 28 eyes, representing a prevalence of 7.3%. Among patients with pauci- or oligo-articular forms of the disease, the percentage increased to 13.3%; polyarticular forms accounted for 10%. Only one of the 12 patients with psoriatic arthritis developed uveitis. Mean age at diagnosis of the ocular condition was 4.5 years and the interval between diagnosis of arthritis to detection of uveitis was 661.5 months. In two patients uveitis was diagnosed before arthritis. Thirty-seven episodes of uveitic activity were identified, of which 27 were asymptomatic. Fifty-three percent of the affected eyes developed complications (posterior synechias in 43%, cataracts in 25%, in-band keratopathy in 18% and glaucoma in 7%). Surgery was required in six eyes. A marked loss of vision occurred in four eyes, despite ophthalmologic treatment. Conclusions Anterior uveitis is a cause of morbidity in JIA. Periodic ophthalmologic explorations are essential for early diagnosis and treatment.

Uveítis y artritis idiopática juvenil / Uveitis and juvenile idiopathic arthritis

Objetivo: La uveítis anterior es una de las manifestaciones extraarticulares más importantes de la artritis idiopática juvenil (AIJ). El objetivo de este estudio ha sido analizar la frecuencia de uveítis en pacientes con AIJ y describir sus características clínicas y evolutivas. Pacientes y métodos: Se analizaron los pacientes que presentaron uveítis entre los 234 niños con AIJ diagnosticados en nuestro hospital. Resultados: Presentaron uveítis 17 pacientes, 16 niñas y un niño, en 28 ojos, lo que supone una prevalencia de 7,3% en el total de niños con AIJ. Entre los pacientes con una forma pauciarticular u oligoarticular de la enfermedad el porcentaje se elevó hasta el 13,3% y fue del 10% en las formas poliarticulares. Sólo 1 de los 12 pacientes con artritis psoriásica desarrolló uveítis. La media de edad al diagnóstico de la afectación ocular fue de 4,5 años y el tiempo transcurrido entre el diagnóstico de la artritis y el de la uveítis fue de 661,5 meses. En 2 casos el diagnóstico de uveítis precedió al de artritis. Se objetivaron 37 episodios de actividad de la uveítis de los que 27 fueron asintomáticos. En el 53% de los ojos afectados aparecieron complicaciones (sinequias posteriores 43%, cataratas 25%, queratopatía en banda 18% y glaucoma 7%). Requirieron cirugía 6 ojos y 4 ojos presentaron una pérdida grave de visión a pesar del tratamiento oftalmológico. Conclusiones: La uveítis anterior es una causa de morbilidad en la AIJ. La exploración oftalmológica periódica en estos pacientes es obligada para establecer el diagnóstico y tratamiento precoces (AU)

[Treatment of idiopathic juvenile arthritis with intraarticular triamcinolone acetonide injections]. / Tratamiento de la artritis idiopática juvenil con infiltraciones articulares de acetónido de triamcinolona.

AIM: To evaluate the therapeutic response to intraarticular triamcinolone acetonide injections in patients with juvenile idiopathic arthritis. METHODS: Eight-eight patients were prospectively evaluated after receiving one or more intraarticular triamcinolone acetonide injections. A total of 194 joints were injected: 68 joints in 39 children with oligoarticular onset juvenile idiopathic arthritis, 36 joints in 17 children with polyarticular onset, 67 joints in 20 children with systemic onset, and 23 joints in 12 children with spondyloarthropathy. RESULTS: Full resolution of signs of inflammation was achieved in 131 of 194 joints (67.5%). The percentage of remission was significantly lower in patients with systemic onset of the disease (36% versus 80% in the other groups). At the 6-month follow-up, 70% of the joints remained in remission. No significant complications were observed. CONCLUSIONS: Intraarticular triamcinolone injections are safe and effective in children with chronic arthritis.