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OBJECTIVES: To define disease activity measures, muscle strength and functional assessments in new-onset juvenile dermatomyositis (JDM) patients, at disease onset and follow up. METHODS: A registry was set up in 18 hospitals, enrolling patients over 3-years (2015-2018). Clinical assessments were performed at baseline, and at 6, 12, 18 and 24 months after diagnosis. Disease Activity Score (DAS20), skin and musculoskeletal DAS sub-scales; Manual Muscle Test (MMT8); Childhood Myositis Assessment Scale (CMAS); Childhood Health Assessment Questionnaire disability index (CHAQ_DI 0-3) and 10 cm Visual Analog Scale (VAS) for overall wellbeing scores were compared by Poisson Model and Wald post-test for repeated measures. RESULTS: Ninety-six cases, being 61 (64%) females, median age 10 years had JDM diagnosis and 12 (13%) onset calcinosis. Mean ±SD scores at diagnosis and 6 months intervals for DAS20 (0-20) were 7.8±5, 6.3 ±4.8, 5±4, 4.9 ±5 and 0.5 ±2.3; with significant difference from baseline (p<0.01). Skin DAS subscales were 2.8±3.3, 1.8±2.9, 1,1±2.2, 0.6±1.8, 0.4±1.5. MMT (0-80) 62.6±20.4, 70.2±13.5, 73.3±11, 75.7±7.9 and 74.8±7.8, with significant difference from baseline up to 6 months (p=0.016); CMAS (0-53) 29.5±11.4, 33.1±8.3, 34.2±5.8, 34±6 and 33.3±5.4. CHAQ-DI (0-3) 1±0.9, 0.6±0.7, 0.8±0.8, 1±0.8 and 1±0.3; parents VAS 4.1±2.5, 2±2.1; 1.3±2.8, 4.1±3.1, 1.7±2.2. There was no significant difference for CMAS, CHAQ-DI and parents VAS from baseline up to 24-month assessment. CONCLUSIONS: DAS20 scores improved gradually during follow up, MMT8 improved significantly during the first 6 months and CMAS, CHAQ-DI and parents VAS scores had no significant improvement with persistent functional impairment over 2-years.
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BACKGROUND: Rheumatic diseases are associated with an increase in overall risks of tuberculosis (TB). The aim of this study was to evaluate the frequency of TB and the frequency of latent TB infection (LTBI), in clinical practice, for juvenile idiopathic arthritis (JIA) patients from high and low risk of TB incidence endemic countries. METHODS: This is an international, multicenter, cross-sectional, observational study of data collection from Brazil and Registry of Portugal at REUMA.PT. The inclusion criteria were patients with Juvenile Idiopathic Arthritis (JIA) with age ≤ 18 years who underwent screening for Mycobacterium tuberculosis infection [tuberculin skin test (TST) and/or interferon gamma release assay (IGRA)]. Chest X-rays and history of exposure to TB were also assessed. RESULTS: 292 JIA patients were included; mean age 14.3 years, mean disease duration 7.5 years, 194 patients (66.4%) performed only TST, 14 (4.8%) only IGRA and 84 (28.8%) both. The frequency of LTBI (10.6%) and TB was similar between the two countries. The reasons for TB screening were different; in Brazil it was performed more often at JIA onset while in Portugal it was performed when starting Disease Modified Anti-Rheumatic Drugs (DMARD) treatment (p < 0.001). Isoniazid therapy was prescribed in 40 (13.7%) patients (31 with LTBI and 9 with epidemiologic risks and/or due to contact with sick people). Only three patients (1%) developed active TB. CONCLUSION: We found nearly 10% of patients with LTBI, a small percentage of patients with treatment due to epidemiologic risks and only 1% with active TB. Distinct reasons and screening methods for LTBI were observed between the two countries.
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Antirreumáticos , Artritis Juvenil , Tuberculosis Latente , Adolescente , Antirreumáticos/uso terapéutico , Artritis Juvenil/complicaciones , Artritis Juvenil/diagnóstico , Artritis Juvenil/tratamiento farmacológico , Estudios Transversales , Humanos , Ensayos de Liberación de Interferón gamma/métodos , Tuberculosis Latente/diagnóstico , Tuberculosis Latente/tratamiento farmacológico , Tuberculosis Latente/epidemiología , Prueba de Tuberculina/métodosRESUMEN
Abstract Background: Rheumatic diseases are associated with an increase in overall risks of tuberculosis (TB). The aim of this study was to evaluate the frequency of TB and the frequency of latent TB infection (LTBI), in clinical practice, for juvenile idiopathic arthritis (JIA) patients from high and low risk of TB incidence endemic countries. Methods: This is an international, multicenter, cross-sectional, observational study of data collection from Brazil and Registry of Portugal at REUMA.PT. The inclusion criteria were patients with Juvenile Idiopathic Arthritis (JIA) with age ≤ 18 years who underwent screening for Mycobacterium tuberculosis infection [tuberculin skin test (TST) and/or interferon gamma release assay (IGRA)]. Chest X-rays and history of exposure to TB were also assessed. Results: 292 JIA patients were included; mean age 14.3 years, mean disease duration 7.5 years, 194 patients (66.4%) performed only TST, 14 (4.8%) only IGRA and 84 (28.8%) both. The frequency of LTBI (10.6%) and TB was similar between the two countries. The reasons for TB screening were different; in Brazil it was performed more often at JIA onset while in Portugal it was performed when starting Disease Modified Anti-Rheumatic Drugs (DMARD) treatment (p < 0.001). Isoniazid therapy was prescribed in 40 (13.7%) patients (31 with LTBI and 9 with epidemiologic risks and/or due to contact with sick people). Only three patients (1%) developed active TB. Conclusion: We found nearly 10% of patients with LTBI, a small percentage of patients with treatment due to epide-miologic risks and only 1% with active TB. Distinct reasons and screening methods for LTBI were observed between the two countries.
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BACKGROUND: Rheumatic heart disease (RHD) complicating acute rheumatic fever (ARF) remains an important health problem in developing countries. No definitive diagnostic test for ARF exists and the role of Doppler echocardiography (DEC) for long-term prognostic evaluation following ARF is not well established. OBJECTIVE: To investigate the prognostic value of DEC in patients with ARF as a predictor of chronic valve dysfunction. METHODS: Prospectively enrolled patients with clinical ARF had a DEC performed soon after diagnosis and repeated at 1, 3, 6 and 12 months and thereafter at every 1-2 years. We defined chronic valve dysfunction by ≥ 3 of the following: increased valve thickening, commissure fusion, subvalvular thickening, reduced leaflet mobility, non-trivial mitral and/or aortic regurgitation. We performed univariate analysis and developed multivariate logistic regression models to identify variables that may influence evolution to RHD. p <0.05 was considered significant. RESULTS: We evaluated 70(57% men) patients, 10.8±5.6 years-old during the ARF episode and followed for 95±26 months. Chronic valve dysfunction was identified in 36(51.4%) which fulfilled criteria for RHD and 10(27.8%) of them died or underwent valve surgery. Univariate analysis showed that mitral valve thickening and presence of mitral regurgitation at baseline DEC, were associated with RHD(p<0.01). Multivariate logistic regression showed that only mitral valve thickness either as a continuous (Odds-Ratio:5.8;95%CI:1.7-19.7) or as a categorical variable (Odds-Ratio:4.04;95%CI:1.06-15.3) was an independent predictor of chronic valve dysfunction. CONCLUSIONS: Mitral leaflets thickening documented at the time of diagnosis of ARF is a consistent prognostic marker for the subsequent evolution to RHD.
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Válvula Mitral/fisiología , Fiebre Reumática/fisiopatología , Adolescente , Niño , Preescolar , Femenino , Humanos , Modelos Logísticos , Masculino , Insuficiencia de la Válvula Mitral/fisiopatología , Análisis Multivariante , Estudios Prospectivos , Cardiopatía Reumática/fisiopatologíaRESUMEN
BACKGROUND: Systemic lupus erythematosus (SLE) is an independent risk factor for cardiovascular events. The present study determined the prevalence of subclinical atherosclerosis in childhood-onset SLE using the carotid intima-media thickness (CIMT) measurement and investigated associations between traditional and nontraditional risk factors for atherosclerosis, such as medications, SLE Disease Activity Index - SLEDAI-2 K and SLICC-ACR damage index and CIMT. METHODS: Cross-sectional prospective study between 2017 and 2018. CIMT was assessed by ultrasonography. Data were collected by chart review, nutritional evaluation and laboratory tests and analyzed by Fisher, Wilcoxon-Mann-Whitney tests, multiple linear and log binomial regression. RESULTS: Twenty-eight patients (mean age 13.9 years, SD 3) were enrolled. The prevalence of subclinical atherosclerosis was 32% (95% CI 14.8, 49.4). The mean CIMT was 0.43 ± 0.035 mm. The most common traditional risk factors observed were dyslipidemia (82.1%), uncontrolled hypertension (14.2%), obesity (14.3%), and poor diet (78.6%). Uncontrolled hypertension (p = 0.04), proteinuria (p = 0.02), estimated glomerular filtration rate < 75 ml /min/1.73 m2 (p = 0.02) and SLEDAI-2 K > 5 (P = 0.04) were associated with subclinical atherosclerosis. SLEDAI-2 K > 5 maintained association with CIMT after adjusting for control variables. CONCLUSION: Subclinical atherosclerosis is frequently observed in cSLE, mainly in patients with moderate to severe disease activity.
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Aterosclerosis/etiología , Lupus Eritematoso Sistémico/complicaciones , Adolescente , Aterosclerosis/diagnóstico , Aterosclerosis/epidemiología , Grosor Intima-Media Carotídeo , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Prevalencia , Estudios Prospectivos , Factores de Riesgo , Adulto JovenRESUMEN
Juvenile-Takayasu arteritis (j-TA) is a difficult diagnosis and some patients develop uncommon manifestations and associated diseases that may contribute to the delayed diagnosis. Our aim was to identify the misdiagnoses, the associated diseases and the atypical manifestations observed in a j-TA Brazilian multicentre study. 71 children and adolescents who met the classification criteria for j-TA were included. The misdiagnoses, the associated diseases and the atypical manifestations were evaluated. 19 (26.8%) patients had misdiagnoses. The most common of them was aortic coarctation in six (8.4%) patients, followed by rheumatic fever in five (7.0%) and one patient presented with both former diagnoses. Limb pain (two patients), spondyloarthropathy, juvenile idiopathic arthritis (JIA), spinal arteriovenous malformation, polyarteritis nodosa (PAN) and fever of unknown origin (FUO) were other misdiagnoses. Patients who had misdiagnoses previously to j-TA diagnosis presented a trend to have a longer diagnosis delay. 11 (15.5%) patients had 14 TA-associated diseases, such as pulmonary tuberculosis (5 patients), rheumatic fever (2 patients), spondyloarthropathy, polyarticular JIA, Crohn's disease, Prader-Willi disease, diabetes mellitus, Moyamoya and primary immunodeficiency. 7 (9.9%) patients presented 10 atypical manifestations, such as pyoderma gangrenosum, erythema nodosum, myositis, chorea, enthesitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities. Our study emphasizes the main misdiagnoses, associated diseases and atypical manifestations that occur in patients with j-TA and warns of the features that may alert paediatricians to this diagnosis, such as constitutional symptoms and elevated inflammatory markers.
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Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico , Adolescente , Brasil , Niño , Diagnóstico Diferencial , Errores Diagnósticos , Femenino , Humanos , Masculino , Poliarteritis Nudosa , Estudios RetrospectivosRESUMEN
BACKGROUND: The effect of topical mometasone for adenoid hypertrophy treatment is well established. Nevertheless, the influence of atopy on this treatment remains ill defined. OBJECTIVE: This study aims to compare the effects of topical mometasone furoate treatment on the adenoid tissue between atopic and nonatopic children. METHODS: Fifty-one children with obstructive respiratory complaints underwent a semistructured clinical questionnaire on nasal symptoms, prick test, and nasoendoscopy. Nasoendoscopic images were digitalized, and both adenoid and nasopharyngeal areas were measured in pixels; the relative adenoid/nasopharyngeal area was calculated. Patients were initially treated for 40 days with nasal saline solution. In a subsequent 40-day period, topical mometasone furoate (total dose, 100 µg/day) was used. RESULTS: Topical mometasone significantly improved nasal obstruction, snoring, and apnea and also significantly reduced the adenoid tissue area related to the nasopharynx (p < 0.0001). Treatment with this glucocorticoid was not influenced by atopy, neither for symptoms nor for adenoid area. CONCLUSION: Topical mometasone furoate significantly reduced the adenoid tissue area and led to a supplementary improvement of nasal symptoms. This improvement was similar for atopic and nonatopic patients.
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Tonsila Faríngea/patología , Antiinflamatorios/uso terapéutico , Pregnadienodioles/uso terapéutico , Administración Tópica , Niño , Femenino , Humanos , Hipertrofia , Masculino , Furoato de Mometasona , Pregnadienodioles/administración & dosificaciónRESUMEN
BACKGROUND: Immunoglobulin E (IgE) reactivity to individual allergens among cockroach-allergic patients has revealed wide variability. The aim of this study was to assess the effectiveness of recombinant cockroach allergens for skin testing, and to determine sensitization profiles among cockroach-allergic patients living in Brazil. METHODS: Fifty-seven cockroach-allergic patients with asthma and/or rhinitis were recruited. Skin testing with recombinant (r) allergens from Periplaneta americana (rPer a 1 and rPer a 7) and Blattella germanica (rBla g 2, rBla g 4 and rBla g 5) were performed at 10 µg/ml and 5 µg/ml (rPer a 1). IgE antibodies to rPer a 7 and rPer a 1 were quantitated by ELISA. RESULTS: Of 57 patients tested, 3 (5.3%), 24 (42.1%), 4 (7%), 3 (5.3%) and 4 (7%) showed positive reactions to rPer a 1, rPer a 7, rBla g 2, rBla g 4 and rBla g 5, respectively. Twenty-eight patients (49.1%) had positive tests to at least one allergen. In keeping with skin test results, 31/57 patients (54.4%) and 5/55 patients (9%) had detectable IgE to rPer a 7 and rPer a 1, respectively. Levels of IgE to rPer a 7 were higher in patients with positive tests to rPer a 7 than those with negative tests (geometric mean 13.2 and 1.8 IU/ml, p < 0.05). There was good concordance of results of skin tests and measurements of serum IgE to rPer a 7. CONCLUSION: IgE reactivity to rPer a 7 (P. americana tropomyosin) was dominant among patients in Brazil. However, 50% of the patients did not present reactivity to any of the recombinant allergens tested.
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Alérgenos , Asma , Blattellidae/inmunología , Periplaneta/inmunología , Proteínas Recombinantes , Rinitis , Pruebas Cutáneas , Adolescente , Adulto , Alérgenos/inmunología , Animales , Asma/complicaciones , Asma/diagnóstico , Niño , Femenino , Humanos , Inmunoglobulina E/sangre , Inmunoglobulina E/inmunología , Masculino , Persona de Mediana Edad , Proteínas Recombinantes/inmunología , Rinitis/complicaciones , Rinitis/diagnósticoRESUMEN
BACKGROUND: Acute respiratory infections (ARI) are frequent in children and complications can occur in patients with chronic diseases. We evaluated the frequency and impact of ARI and influenza-like illness (ILI) episodes on disease activity, and the immunogenicity and safety of influenza vaccine in a cohort of juvenile idiopathic arthritis (JIA) patients. METHODS: SURVEILLANCE OF RESPIRATORY VIRUSES WAS CONDUCTED IN JIA PATIENTS DURING ARI SEASON (MARCH TO AUGUST) IN TWO CONSECUTIVE YEARS: 2007 (61 patients) and 2008 (63 patients). Patients with ARI or ILI had respiratory samples collected for virus detection by real time PCR. In 2008, 44 patients were immunized with influenza vaccine. JIA activity index (ACRPed30) was assessed during both surveillance periods. Influenza hemagglutination inhibition antibody titers were measured before and 30-40 days after vaccination. RESULTS: During the study period 105 ARI episodes were reported and 26.6% of them were ILI. Of 33 samples collected, 60% were positive for at least one virus. Influenza and rhinovirus were the most frequently detected, in 30% of the samples. Of the 50 JIA flares observed, 20% were temporally associated to ARI. Influenza seroprotection rates were higher than 70% (91-100%) for all strains, and seroconversion rates exceeded 40% (74-93%). In general, response to influenza vaccine was not influenced by therapy or disease activity, but patients using anti-TNF alpha drugs presented lower seroconversion to H1N1 strain. No significant differences were found in ACRPed30 after vaccination and no patient reported ILI for 6 months after vaccination. CONCLUSION: ARI episodes are relatively frequent in JIA patients and may have a role triggering JIA flares. Trivalent split influenza vaccine seems to be immunogenic and safe in JIA patients.
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OBJECTIVE: Chronic rhinitis and adenoid hypertrophy are the main causes of nasal obstruction in children and proper treatment of these factors seem essential for controlling nasal obstructive symptoms. This study aims to evaluate the effects of topical mometasone treatment on symptoms and size of adenoid tissue in children with complaints of nasal obstruction and to compare this approach to continuous nasal saline douching plus environmental control alone. METHODS: Fifty-one children with nasal obstructive complaints were submitted to a semi-structured clinical questionnaire on nasal symptoms, prick test and nasoendoscopy. Nasoendoscopic images were digitalized, and both adenoid and nasopharyngeal areas were measured in pixels. The relation adenoid/nasopharyngeal area was calculated. Patients were subsequently re-evaluated in two different periods: following 40 days of treatment with nasal douching and environmental prophylaxis alone; and after an subsequent 40 day-period, when topical mometasone furoate (total dose: 100µg/day) was superposed. RESULTS: Nasal symptoms and snoring significantly improved after nasal douching, and an additional gain was observed when mometasone furoate was included to treatment. Saline douching did not influence the adenoid area, whereas a significant reduction on adenoid tonsil was observed after 40 days of mometasone treatment (P<0.0001). CONCLUSION: Nasal saline douching significantly improved nasal symptoms without interfering in adenoid dimension. In contrast, mometasone furoate significantly reduced adenoid tissue, and led to a supplementary improvement of nasal symptoms.
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Tonsila Faríngea/patología , Obstrucción Nasal/tratamiento farmacológico , Pregnadienodioles/uso terapéutico , Rinitis/tratamiento farmacológico , Irrigación Terapéutica/métodos , Tonsila Faríngea/efectos de los fármacos , Administración Intranasal , Antiinflamatorios/uso terapéutico , Niño , Preescolar , Enfermedad Crónica , Estudios de Cohortes , Ambiente , Femenino , Estudios de Seguimiento , Humanos , Hipertrofia/complicaciones , Hipertrofia/tratamiento farmacológico , Masculino , Furoato de Mometasona , Obstrucción Nasal/etiología , Variaciones Dependientes del Observador , Rinitis/complicaciones , Rinitis/diagnóstico , Medición de Riesgo , Cloruro de Sodio/uso terapéutico , Estadísticas no Paramétricas , Resultado del TratamientoRESUMEN
INTRODUCTION/OBJECTIVES: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. MATERIAL AND METHODS: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. RESULTS: Vaccination card was seen by 100% of the professionals at the first visit and by 36% annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44%, 64%, and 48%, respectively. The professionals were divided into two groups: Group A (≤ 15 years of practice, n = 12) and B (≥ 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). CONCLUSIONS: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.
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Pediatría , Pautas de la Práctica en Medicina , Enfermedades Reumáticas , Reumatología , Vacunación/estadística & datos numéricos , Niño , HumanosRESUMEN
INTRODUÇÃO/OBJETIVOS: Avaliar a prática clínica com relação à verificação do cartão vacinal e à indicação de vacinas específicas em pacientes com doenças reumáticas pediátricas em uso de diferentes drogas, e evidenciar a possível associação entre frequência de vacinação e tempo de prática clínica dos reumatologistas pediátricos do estado de São Paulo. MATERIAL E MÉTODOS: Um questionário foi enviado para os reumatologistas pediátricos do Departamento de Reumatologia da Sociedade de Pediatra de São Paulo. Esse instrumento incluiu questões sobre tempo de prática em Reumatologia Pediátrica, vacinação de pacientes com Lúpus Eritematoso Sistêmico Juvenil (LESJ), artrite idiopática juvenil (AIJ), dermatomiosite juvenil (DMJ) e imunização de acordo com os tratamentos utilizados. RESULTADOS: Cartão de vacinação foi visto por 100 por cento dos profissionais na primeira consulta e por 36 por cento anualmente. Vacinas de agentes vivos não foram recomendadas para pacientes com LESJ, AIJ e DMJ em 44 por cento, 64 por cento e 48 por cento, respectivamente. Os profissionais foram divididos em dois grupos: A (< 15 anos de prática, n = 12) e B (> 16 anos, n = 13). Nenhuma diferença estatística foi observada no uso de vacinas de agentes vivos e vacinas de agentes inativos ou componentes proteicos em relação ao tratamento nos dois grupos (P > 0,05). Além disso, os grupos foram similares em relação à opinião sobre a gravidade de imunossupressão em pacientes com LESJ, AIJ e DMJ com ou sem atividade e a terapêutica utilizada (P > 0,05). CONCLUSÕES: A frequência de vacinação por reumatologistas pediátricos de São Paulo é baixa, especialmente após a primeira consulta, e não é influenciada pelo tempo de prática profissional.
INTRODUCTION/OBJECTIVES: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. MATERIAL AND METHODS: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. RESULTS: Vaccination card was seen by 100 percent of the professionals at the first visit and by 36 percent annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44 percent, 64 percent, and 48 percent, respectively. The professionals were divided into two groups: Group A (< 15 years of practice, n = 12) and B (> 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). CONCLUSIONS: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.
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Niño , Humanos , Pediatría , Pautas de la Práctica en Medicina , Enfermedades Reumáticas , Reumatología , Vacunación/estadística & datos numéricosRESUMEN
Crianças e adolescentes com doenças reumatológicas apresentam maior prevalência de doenças infecciosas quando comparados com a população em geral, em decorrência de atividade da doença, possível deficiência imunológica secundária à própria doença, ou uso de terapia imunossupressora. A vacinação é uma medida eficaz para a redução da morbidade e mortalidade nesses pacientes. O objetivo deste artigo foi realizar um consenso de eficácia e segurança das vacinas em crianças e adolescentes com doenças reumatológicas infantis baseadas em níveis de evidência científica. Imunização passiva para os pacientes e orientações para as pessoas que convivem com doentes imunodeprimidos também foram incluídas. Os 32 pediatras reumatologistas membros do Departamento de Reumatologia da Sociedade de Pediatria de São Paulo (SPSP) e/ou da Comissão de Reumatologia Pediátrica da Sociedade Brasileira de Reumatologia elaboraram o consenso, sendo que alguns desses profissionais estão envolvidos em pesquisas e publicações científicas nesta área. A pesquisa dos termos eficácia e/ou segurança das diferentes vacinas em crianças e adolescentes com doenças reumatológicas foi realizada nas bases de Medline e Scielo, de 1966 até março de 2009, incluindo revisões, estudos controlados e relatos de casos. O grau de recomendação e o nível científico de evidências dos estudos foram classificados em quatro níveis para cada vacina. De um modo geral, as vacinas inativadas e de componentes são seguras nos pacientes com doenças reumatológicas, mesmo em uso de terapias imunossupressoras. Entretanto, vacinas com agentes vivos atenuados são, em geral, contraindicadas para os pacientes imunossuprimidos.
Incidence of infectious diseases is higher in children and adolescents with rheumatic diseases than in the general population due to disease activity, possible immune deficiency secondary to the disease itself, or the use of immunosuppressive drugs. Vaccination is effective in reducing morbidity and mortality in those patients. The objective of this study was to establish an evidence-based consensus on the efficacy and safety of vaccination in children and adolescents with rheumatic diseases. Passive immunization of patients and guidelines for people who live with immunosuppressed patients were also included. The 32 pediatric rheumatologists of the Rheumatology Department of the Pediatrics Society of São Paulo, (SPSP, from the Portuguese), São Paulo, SP, Brazil, and/or the Commission on Pediatrics Rheumatology of the Brazilian Society of Rheumatology are responsible for this consensus; some of those professionals are involved on research and scientific publications in this field. The words efficacy and/or safety of different vaccines in children and adolescents with rheumatologic diseases were searched in Medline and Scielo data bases from 1966 to March 2009, including reviews, controlled studies, and case reports. The degree of recommendation and the scientific evidence of the studies were classified in four levels for each vaccine. As a rule, inactive and protein components vaccines are safe for patients with rheumatologic diseases, even in the presence of immunosuppressive therapy. However, live attenuated vaccines are, in general, contraindicated for immunosuppressed patients.
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Humanos , Niño , Adolescente , Artritis Juvenil , Consenso , Inmunización Pasiva , Lupus Eritematoso Sistémico , Enfermedades Reumáticas , Vacunación , VacunasRESUMEN
BACKGROUND: Evidence indicates that infection with Ascaris lumbricoides may promote development of allergy and asthma. OBJECTIVE: To study the role of tropomyosin, a pan-allergen in invertebrates, in IgE responses to A lumbricoides. METHODS: Recombinant A lumbricoides and Periplaneta americana tropomyosins were expressed in Pichia pastoris. Levels of IgE to tropomyosins from A lumbricoides and P americana were determined by chimeric ELISA in sera from 119 children living in a parasite-endemic area and 112 patients with cockroach allergy from the allergy clinics. Presence of tropomyosin in A lumbricoides larvae at L3 stage was evaluated by immunofluorescence using mAb 1A6, directed against mite tropomyosin. Molecular modeling of P americana and A lumbricoides tropomyosins was performed by using the MODELLER program. RESULTS: A lumbricoides tropomyosin showed 69% to 98% sequence identity to tropomyosins from other invertebrates. The predicted structure of A lumbricoides tropomyosin was similar to that of P americana tropomyosin and showed the characteristic coiled-coil structure. Strong correlation was found for IgE antibodies to tropomyosins from A lumbricoides and P americana in sera from children living in a parasite-endemic area and from patients with cockroach allergy. Larvae of A lumbricoides reacted strongly with mAb 1A6. CONCLUSION: Tropomyosin induces IgE responses in A lumbricoides-infected children and in patients allergic to cockroach.
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Ascaris lumbricoides/inmunología , Inmunoglobulina E/metabolismo , Periplaneta/inmunología , Tropomiosina/inmunología , Tropomiosina/metabolismo , Adolescente , Adulto , Secuencia de Aminoácidos , Animales , Ascaris lumbricoides/química , Asma/inmunología , Asma/metabolismo , Niño , Preescolar , Reacciones Cruzadas , Humanos , Hipersensibilidad/inmunología , Hipersensibilidad/metabolismo , Inmunoglobulina E/biosíntesis , Persona de Mediana Edad , Datos de Secuencia Molecular , Periplaneta/química , Tropomiosina/químicaRESUMEN
Deficiencies of complement proteins of the classical pathway are strongly associated with the development of autoimmune diseases. Deficiency of C1r has been observed to occur concomitantly with deficiency in C1s and 9 out of 15 reported cases presented systemic lupus erythematosus (SLE). Here, we describe a family in which all four children are deficient in C1s but only two of them developed SLE. Hemolytic activity mediated by the alternative and the lectin pathways were normal, but classical pathway activation was absent in all children's sera. C1s was undetectable, while in the parents' sera it was lower than in the normal controls. The levels of C1r observed in the siblings and parents sera were lower than in the control, while the concentrations of other complement proteins (C3, C4, MBL and MASP-2) were normal in all family members. Impairment of C1s synthesis was observed in the patients' fibroblasts when analyzed by confocal microscopy. We show that all four siblings are homozygous for a mutation at position 938 in exon 6 of the C1s cDNA that creates a premature stop codon. Our investigations led us to reveal the presence of previously uncharacterized splice variants of C1s mRNA transcripts in normal human cells. These variants are derived from the skipping of exon 3 and from the use of an alternative 3' splice site within intron 1 which increases the size of exon 2 by 87 nucleotides.
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Empalme Alternativo , Complemento C1s/deficiencia , Lupus Eritematoso Sistémico/genética , Adulto , Secuencia de Bases , Células Cultivadas , Niño , Complemento C1s/genética , Complemento C1s/inmunología , Exones , Femenino , Fibroblastos/inmunología , Humanos , Intrones , Lupus Eritematoso Sistémico/inmunología , Masculino , Datos de Secuencia Molecular , Linaje , ARN Mensajero/genética , ARN Mensajero/inmunologíaRESUMEN
A síndrome de Bloom é um raro distúrbio de instabilidade cromossômica, devido a defeitos de reparo do DNA. O gene responsável BLMfoi mapeado no cromossomo 15q e causa redução significante da expressão da enzima DNA-helicase no núcleo celular. Esta enzima é importante para os mecanismos de reparo do DNA. Pacientes geralmente apresentam elevada suscetibilidade para desenvolvimento de câncer e infecções, bacterianas recorrentes. Nós avaliamos o sistema imunológico de dois irmãos (menino de 13 anos e menina de 6 anos) com síndrome de Bloom, os quais tinham similar suscetibilidade para infecções respiratórias recorrentes, mas diferentes anormalidades imunológicas. O menino apresentou níveis persistentemente baixos de IgM e de células CD4+. Os níveis de IgA diminuíram quando ele tinha 11 anos. A menina apresentou níveis persistentemente baixos dos níveis de IgM e IgA, e não respondeu à vacina polissacarídica. Os níveis de IgG diminuíram quando ela tinha 6 anos. As diferentes anormalidades imunológicas são discutidas
Asunto(s)
Niño , Adolescente , Formación de Anticuerpos , Síndrome de Bloom , Deficiencia de IgA , Sistema Inmunológico , Fenotipo , Inmunodeficiencia Combinada Grave , Inmunidad Celular , Técnicas y Procedimientos DiagnósticosRESUMEN
The prevalence and clinical relevance of IgM and IgA RF detected by ELISA were studied in 91 patients with juvenile rheumatoid arthritis (JRA) and 45 healthy children. IgM and IgA RF were detected, respectively, in 33 and 44% of the patients, compared to 6.7 and 15.6% of the healthy children (p = 0.001 and 0.0006, respectively). The frequency of IgM RF was significantly higher in patients with polyarticular (52%) as compared to systemic onset JRA (21%; p = 0.04). Five out of ninety-one patients and none of the control group were IgM RF positive by the latex test. High levels of IgM RF were detected more frequently in patients with active disease (p = 0.01) and positive latex agglutination test (p < 0.001) and had a marginally significant association with severe radiological deformities (p = 0.05). The presence of IgA RF was associated with active disease in polyarticular onset JRA children (p = 0.04). In conclusion, high levels of IgM RF and the detection of IgA RF can be useful in assessing clinical activity in a subset of patients with JRA.
Asunto(s)
Artritis Juvenil/sangre , Artritis Juvenil/patología , Factor Reumatoide/sangre , Adolescente , Adulto , Biomarcadores/sangre , Estudios de Casos y Controles , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Inmunoglobulina A/sangre , Inmunoglobulina M/sangre , Pruebas Inmunológicas/métodos , Masculino , Factor Reumatoide/inmunologíaRESUMEN
The objective of this study was to evaluate risk factors for persistent wheezing in a group of 2-4-year-old children after an index-wheezing episode in infancy. Eighty infants who had been seen at the Emergency Department for an episode of acute wheezing were followed for 2 yr in this prospective study. Caregivers completed a questionnaire, and children underwent clinical evaluation and skin prick testing 2 yr following the index-wheezing episode. Detection of respiratory viruses and analysis of exposure to major indoor allergens were carried out at enrollment. Immunoglobin E antibodies were measured at the beginning of the study and at the end of follow-up, using the CAP system. Logistic regression analysis was performed to identify factors associated with persistent wheezing. Seventy-three children (44 boys) completed the study. After 2 yr, 38 (52%) reported three or more wheezing episodes in the past 12 months (persistent wheezers). Independent risk factors for persistence of wheezing were allergic sensitization and exposure to cockroach allergen in the kitchen. Breast-feeding for at least 1 month was a protective factor. A strong association between allergic sensitization and persistence of wheezing was found in a group of very young children living in a subtropical area.
Asunto(s)
Alérgenos/administración & dosificación , Lactancia Materna , Cucarachas/inmunología , Hipersensibilidad/etiología , Ruidos Respiratorios/etiología , Animales , Brasil , Preescolar , Femenino , Humanos , Hipersensibilidad/inmunología , Hipersensibilidad/prevención & control , Lactante , Masculino , Estudios Prospectivos , Ruidos Respiratorios/inmunología , Factores de RiesgoRESUMEN
Alergia a barata é um importante fator de risco para asma em todo o mundo, inclusive no Brasil. Evidências indicam que a combinação de sensibilização a barata e exposição a alérgenos de barata no domicílio está associada a asma mais grave. Essas observações sugerem que alérgenos de barata são particularmente potentes em induzir resposta IgE. Estratégias para diminuir a exposição ambiental a alérgenos de barata requerem limpeza extensa, educação e extermínio profissional de insetos, que podem ser difíceis de manter. Um dos principais alérgenos de barata, tropomiosina, é compartilhado com ácaros, camarão e outros crustáceos e moluscos, e com o parasitaintestinal Ascaris lumbricoides. Análise da identidade de seqüências e da estrutura tridimensional obtida por modelagem dessas moléculas permite-nos formular a hipótese de que reatividade cruzada IgE para tropomiosina poderia ter relevância clínica e ter um papel na imunomodulação de parasitas sobre o desenvolvimento de alergia e asma. Estudo futuros investigando novas estratégias para diagnóstico e tratamento de alergia a barata podem incluir o uso de alérgenos recombinantes para testes in vivo e in vitro, e imunoterapia alérgeno-especifica, que poderá beneficiar pacientes com asma e/ou rinite alérgicos a esse inseto.
Asunto(s)
Niño , Humanos , Alérgenos , Ascaris lumbricoides , Asma , Clonación Molecular , Cucarachas , Técnicas In Vitro , Enfermedades Parasitarias , Periplaneta , Tropomiosina , Métodos , Técnicas y Procedimientos DiagnósticosRESUMEN
BACKGROUND: Risk factors for acute wheezing among children in subtropical areas are largely unknown. OBJECTIVE: To investigate the role of viral infections, allergen sensitization, and exposure to indoor allergens as risk factors for acute wheezing in children 0 to 12 years old. METHODS: One hundred thirty-two children 0 to 12 years of age who sought emergency department care for wheezing and 65 children with no history of wheezing were enrolled in this case-control study. Detection of respiratory syncytial virus antigen, rhinovirus and coronavirus RNA, adenovirus, influenza, and parainfluenza antigens was performed in nasal washes. Total IgE and specific IgE to mites, cockroach, cat, and dog were measured with the CAP system. Major allergens from mites, cockroach, cat, and dog were quantified in dust samples by ELISA. Univariate and multivariate analyses were performed by logistic regression. RESULTS: In children under 2 years of age, infection with respiratory viruses and family history of allergy were independently associated with wheezing (odds ratio, 15.5 and 4.2; P = .0001 and P = .008, respectively). Among children 2 to 12 years old, sensitization to inhalant allergens was the major risk factor for wheezing (odds ratio, 2.7; P = .03). High-level allergen exposure, exposure to tobacco smoke, and lack of breast-feeding showed no association with wheezing. CONCLUSIONS: Some risk factors for wheezing previously identified in temperate climates were present in a subtropical area, including respiratory syncytial virus infection in infants and allergy in children older than 2 years. Rhinovirus was not associated with wheezing and did not appear to be a trigger for asthma exacerbations.