[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.

[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.

[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.

[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.

[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.

[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.

[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.

[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.

[Juvenile spondyloarthropathies: descriptive study of 40 patients]. / Espondiloartropatías juveniles: estudio descriptivo de 40 pacientes.

OBJECTIVE: The objective of this study was to describe the clinical characteristics of children with spondyloarthropathies diagnosed in our unit. PATIENTS AND METHODS: We analyzed the patients with SEA syndrome, undifferentiated spondyloarthropathy, psoriatic arthritis, arthritis associated with inflammatory bowel disease and reactive arthritis with onset symptoms before the age of 16. RESULTS: Forty patients were diagnoses as suffering from spondyloarthropathy (29 boys and 11 girls). Eight presented a SEA syndrome, 8 undifferentiated spondyloarthropathy, 8 psoriatic arthritis, 6 arthritis associated with inflammatory bowel disease and 10 with reactive arthritis. No patient met ankylosing spondylitis criteria. In ten children the initial diagnosis was chronic juvenile arthritis. The articular disease had a relapsing and remitting course in one third of the patients. Almost 50% had only one episode and 7 patients had one prolonged course. In general, the functional outcome was good. CONCLUSIONS: Juvenile spondyloarthropathies are a group of rheumatic diseases with common clinical characteristics and differ from chronic juvenile arthritis. Their early recognition is important with regard to treatment and prognosis.

[Intra-articular steroids in chronic juvenile arthritis]. / Esteroides intraarticulares en la artritis crónica juvenil.

The use of intraarticular steroids was evaluated in 15 children with chronic pauciarticular juvenile arthritis. Of the 25 joints injected, 23 responded favorably. At the 6-month check-up, 65% remained in remission. One year after injection, 42% maintained a good response. The beneficial effect of the injection was not associated with age, sex, erythrosedimentation or arthritis duration. A small atrophic lesion in the area of the injection in one patient was the only complication.

[Rheumatic fever and post-streptococcal arthritis. Clinical review]. / Fiebre reumática y artritis postestreptocócicas. Revisión clínica.

We report 11 patients diagnosed of Acute Rheumatic Fever (ARF), and 7 of Poststreptococcal Reactive Arthritis (PSRA): patients with group A beta hemolytic streptococcal infection and articular disease who do not fulfill the modified Jones criteria. All patients with ARF were treated with monthly prophylaxis (with penicillin G benzathine). Carditis was seen in five patients, but only one of them has developed a mitral valve insufficiency. The prophylaxis in patients with PSRA was individualized. None of these patients had clinical evidence of carditis during the acute disease, but one of them developed a mitral and aortic stenosis two years after the initial episode. The possible indication of penicillin prophylaxis in these patients with PSRA in suggested.