Assuntos
Betacoronavirus/patogenicidade , Infecções por Coronavirus/metabolismo , Peptidil Dipeptidase A/metabolismo , Pneumonia Viral/metabolismo , Enzima de Conversão de Angiotensina 2 , Doenças Assintomáticas , COVID-19 , Criança , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/fisiopatologia , Humanos , Imunidade Inata , Pandemias , Pneumonia Viral/imunologia , Pneumonia Viral/fisiopatologia , SARS-CoV-2 , Índice de Gravidade de DoençaAssuntos
Humanos , Criança , Pneumonia Viral/metabolismo , Infecções por Coronavirus/metabolismo , Peptidil Dipeptidase A/metabolismo , Betacoronavirus/patogenicidade , Pneumonia Viral/fisiopatologia , Pneumonia Viral/imunologia , Índice de Gravidade de Doença , Infecções por Coronavirus/fisiopatologia , Infecções por Coronavirus/imunologia , Doenças Assintomáticas , Pandemias , Enzima de Conversão de Angiotensina 2 , SARS-CoV-2 , COVID-19 , Imunidade InataRESUMO
Primary immunodeficiencies (PIDs) represent a large group of diseases that affect all age groups. Although PIDs have been recognized as rare diseases, there is epidemiological evidence suggesting that their real prevalence has been underestimated. We performed an evaluation of a series of 1,008 infants, children, adolescents and adults with well-defined PIDs from a single Brazilian center, regarding age at diagnosis, gender and PID category according to the International Union of Immunological Societies classification. Antibody deficiencies were the most common category in the whole series (61 %) for all age groups, with the exception of <2-year-old patients (only 15 %). In the >30-year-old group, antibody deficiencies comprised 84 % of the diagnoses, mostly consisting of common variable immunodeficiency, IgA deficiency and IgM deficiency. Combined immunodeficiencies represented the most frequent category in <2-years-old patients. Most congenital defects of phagocytes were identified in patients <5 -years of age, as were the diseases of immune dysregulation, with the exception of APECED. DiGeorge syndrome and ataxia-telangiectasia were the most frequent entities in the category of well-defined syndromes, which were mostly identified in patients <10-years of age. Males represented three-quarters and two-thirds of <2 -years-old and 2-5-years -old patients, respectively, whereas females predominated among the >30-year-old patients. Our data indicated that some PIDs were only detected at early ages, likely because affected patients do not survive long. In addition, our data pointed out that different strategies should be used to search for PIDs in infants and young children as compared to older patients.
Assuntos
Síndromes de Imunodeficiência/epidemiologia , Fatores Sexuais , Adolescente , Adulto , Fatores Etários , Brasil , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulina A/genética , Imunoglobulina M/genética , Síndromes de Imunodeficiência/imunologia , Lactente , Recém-Nascido , Masculino , Fagócitos/patologia , Grupos Populacionais , PrevalênciaRESUMO
OBJECTIVES: The aim of the present paper is to evaluate the immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus. METHODS: We performed a prospective and controlled study on a group of 54 SLE patients that were chosen at random to be or not to be vaccinated (28 were vaccinated and 26 were not). Twenty-eight healthy controls, of matching age and sex were also vaccinated. All were submitted to a questionnaire, physical evaluation and laboratory assays: lymphocyte immune-phenotyping by flow cytometry, plasma varicella zoster virus (VZV) serology by ELISA and in vitro interferon gamma (IFNγ) production by T-cells after stimulus with VZV antigen. They were evaluated before vaccination and at 30, 45, 180 and 360 days afterwards. RESULTS: We did not observe any differences in the frequency of adverse events in both vaccinated groups. At study entry, all individuals were seropositive for VZV antibodies. The serum VZV antibody titres similarly increased after vaccination. The frequency of flares and the SLEDAI score were also similar among the patients. Thirty days after vaccination the production of IFNγ specific to VZV was lower in the SLE group compared to healthy controls. In the follow-up we observed 4 cases of herpes zoster in the SLE unvaccinated group, but no zoster in the vaccinated group. CONCLUSIONS: The varicella vaccine was well tolerated in SLE group, who had pre-existing immunity to varicella. The varicella vaccine immunogenicity measurement by serum antibody titres was appropriate. The incidence of HZ was lower in the vaccinated lupus group.
Assuntos
Vacina contra Varicela/imunologia , Herpes Zoster/prevenção & controle , Herpesvirus Humano 3/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Adolescente , Anticorpos Antivirais/sangue , Biomarcadores/sangue , Brasil , Vacina contra Varicela/efeitos adversos , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Feminino , Citometria de Fluxo , Herpes Zoster/imunologia , Herpes Zoster/virologia , Humanos , Imunidade Celular , Imunidade Humoral , Imunofenotipagem/métodos , Interferon gama/sangue , Testes de Liberação de Interferon-gama , Lúpus Eritematoso Sistêmico/sangue , Masculino , Estudos Prospectivos , Linfócitos T/imunologia , Linfócitos T/virologia , Fatores de Tempo , Resultado do TratamentoRESUMO
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.
Assuntos
Pediatria , Padrões de Prática Médica , Doenças Reumáticas , Reumatologia , Vacinação/estatística & dados numéricos , Criança , HumanosRESUMO
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.
Assuntos
Criança , Humanos , Pediatria , Padrões de Prática Médica , Doenças Reumáticas , Reumatologia , Vacinação/estatística & dados numéricosRESUMO
OBJETIVO: Revisar os mecanismos fisiopatológicos e novos alvos terapêuticos, os agentes biológicos disponíveis, principais indicações e a evidência científica atual para o uso de terapias biológicas na população pediátrica. FONTES DE DADOS: Pesquisa na base de dados Medline e SciELO, nas línguas inglesa e portuguesa, entre 2000 e 2009. As palavras-chave usadas foram "agentes biológicos", "crianças" e "adolescentes". SÍNTESE DOS DADOS: Os agentes biológicos são uma importante opção terapêutica para tratar as doenças autoimunes refratárias às terapias convencionais na infância e na adolescência. Com exceção da artrite idiopática juvenil, a maioria dos estudos em outras doenças autoimunes não é controlada. CONCLUSÕES: Os agentes biológicos têm demonstrado eficácia no tratamento de doenças autoimunes pediátricas como artrite idiopática juvenil, miopatias idiopáticas inflamatórias, lúpus eritematoso juvenil, vasculites, uveítes crônicas, doenças inflamatórias intestinais e púrpura trombocitopênica imune crônica, assim como no linfoma não-Hodgkin. Considerando-se o custo elevado e os potenciais eventos adversos, o uso desses agentes deve ser individualizado e acompanhado por especialista.
OBJECTIVE: To review the physiopathology and new therapeutical targets, the available biologic agents, the main indications and the current scientific evidence for the use of biological therapies in the pediatric population. DATA SOURCES: A bibliographical search was obtained from Medline and SciELO databases in English and Portuguese from 2000 to 2009. The key-words included were "biologic agent", "children" and "adolescent". DATA SYNTHESIS: Biologic agents are important therapeutic options to treat refractory autoimmune diseases to conventional therapies in childhood and adolescence. Excluding juvenile idiopathic arthritis, the majority of studies in other autoimmune diseases are uncontrolled trials. CONCLUSIONS: Biologic agents have shown efficacy in the treatment of pediatric autoimmune diseases such as juvenile idiopathic arthritis, idiopathic inflammatory myositis, juvenile systemic lupus erythematosus, vasculitis, chronic uveitis, inflammatory bowel diseases, and chronic immune thrombocytopenic purpura, as well as in non-Hodgkin lymphoma. Considering the high cost and the potential adverse events, the choice to use them must be individualized and followed by a specialist.
Assuntos
Humanos , Criança , Adolescente , Doenças Autoimunes , Terapia BiológicaRESUMO
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.
Assuntos
Humanos , Criança , Adolescente , Artrite Juvenil , Consenso , Imunização Passiva , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Vacinação , VacinasAssuntos
Antirreumáticos/efeitos adversos , Artrite Juvenil/tratamento farmacológico , Imunoglobulina G/efeitos adversos , Síndrome de Ativação Macrofágica/induzido quimicamente , Criança , Quimioterapia Combinada , Etanercepte , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Síndrome de Ativação Macrofágica/tratamento farmacológico , Masculino , Receptores do Fator de Necrose TumoralRESUMO
INTRODUCTION: Associations between systemic lupus erythematosus (SLE) and primary immunodeficiencies (PIDs) were analyzed to gain insight into the physiopathology of SLE. Some PIDs have been consistently associated with SLE or lupus-like manifestations: (a) homozygous deficiencies of the early components of the classical complement pathway in the following decreasing order: in C1q, 93% of affected patients developed SLE; in C4, 75%; in C1r/s, 57%; and in C2, up to 25%; (b) female carriers of X-linked chronic granulomatous disease allele; and (c) IgA deficiency, present in around 5% of juvenile SLE. DISCUSSION: In the first two groups, disturbances of cellular waste-disposal have been proposed as the main mechanisms of pathogenesis. On the other hand and very interestingly, there are PIDs systematically associated with several autoimmune manifestations in which SLE has not been described, such as autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX), and autoimmune lymphoproliferative syndrome (ALPS), suggesting that mechanisms considered as critical players for induction and maintenance of tolerance to autoantigens, such as (1) AIRE-mediated thymic negative selection of lymphocytes, (2) Foxp3+ regulatory T cell-mediated peripheral tolerance, and (3) deletion of auto-reactive lymphocytes by Fas-mediated apoptosis, could not be relevant in SLE physiopathology. The non-description of SLE and neither the most characteristic SLE clinical features among patients with agammaglobulinemia are also interesting observations, which reinforce the essential role of B lymphocytes and antibodies for SLE pathogenesis. CONCLUSION: Therefore, monogenic PIDs represent unique and not fully explored human models for unraveling components of the conundrum represented by the physiopathology of SLE, a prototypical polygenic disease.