ABSTRACT
Abstract This second joint document, written by experts from the Brazilian Association of Allergy and Immunology (ASBAI) and Brazilian Society of Anesthesiology (SBA) concerned with perioperative anaphylaxis, aims to review the pathophysiological reaction mechanisms, triggering agents (in adults and children), and the approach for diagnosis during and after an episode of anaphylaxis. As anaphylaxis assessment is extensive, the identification of medications, antiseptics and other substances used at each setting, the comprehensive data documentation, and the use of standardized nomenclature are key points for obtaining more consistent epidemiological information on perioperative anaphylaxis.
Resumo Este segundo documento, escrito por especialistas da Associação Brasileira de Alergia e Imunologia (ASBAI) e da Sociedade Brasileira de Anestesiologia (SBA) interessados no tema anafilaxia perioperatória, tem por objetivo revisar os mecanismos fisiopatológicos, agentes desencadeantes (em adultos e crianças), assim como a abordagem diagnóstica durante e após o episódio. Por se tratar de uma avaliação abrangente, a identificação das medicações, antissépticos e outras substâncias usadas em cada região, registros detalhados, e nomenclatura padronizada são pontos fundamentais para a obtenção de dados epidemiológicos mais fidedignos sobre a anafilaxia perioperatória.
Subject(s)
Humans , Child , Adult , Drug Hypersensitivity/diagnosis , Drug Hypersensitivity/etiology , Perioperative Period , Anaphylaxis/diagnosis , Anaphylaxis/etiology , Societies, Medical , Vasodilator Agents/adverse effects , In Vitro Techniques , Mastocytosis/complications , Brazil , Preoperative Care , Immunoglobulin E/immunology , Bradykinin/adverse effects , Skin Tests/methods , Risk Factors , IgA Deficiency/complications , Drug Hypersensitivity/physiopathology , Allergy and Immunology , Symptom Assessment , Anaphylaxis/physiopathology , Anesthesiology , Angioedema/chemically induced , Terminology as TopicABSTRACT
Abstract Experts from the Brazilian Association of Allergy and Immunology (ASBAI) and the Brazilian Society of Anesthesiology (SBA) interested in the issue of perioperative anaphylaxis, and aiming to strengthen the collaboration between the two societies, combined efforts to study the topic and to prepare a joint document to guide specialists in both areas. The purpose of the present series of two articles was to report the most recent evidence based on the collaborative assessment between both societies. This first article will consider the updated definitions, treatment and guidelines after a perioperative crisis. The following article will discuss the major etiologic agents, how to proceed with the investigation, and the appropriate tests.
Resumo Especialistas da Associação Brasileira de Alergia e Imunologia (ASBAI) e da Sociedade Brasileira de Anestesiologia (SBA) interessados no tema anafilaxia perioperatória reuniram-se com o objetivo de intensificar a colaboração entre as duas sociedades no estudo desse tema e elaborar um documento conjunto que possa guiar os especialistas de ambas as áreas. O objetivo desta série de dois artigos foi mostrar as evidências mais recentes alicerçadas na visão colaborativa entre as sociedades. Este primeiro artigo versará sobre as definições mais atuais, formas de tratamento e as orientações após a crise no perioperatório. No próximo artigo serão discutidos os principais agentes causais e a condução da investigação com testes apropriados.
Subject(s)
Humans , Child , Adult , Drug Hypersensitivity/diagnosis , Drug Hypersensitivity/etiology , Perioperative Period , Anaphylaxis/diagnosis , Anaphylaxis/etiology , Societies, Medical , Vasodilator Agents/adverse effects , In Vitro Techniques , Mastocytosis/complications , Brazil , Preoperative Care , Immunoglobulin E/immunology , Bradykinin/adverse effects , Skin Tests/methods , Risk Factors , IgA Deficiency/complications , Drug Hypersensitivity/physiopathology , Allergy and Immunology , Symptom Assessment , Anaphylaxis/physiopathology , Anesthesiology , Angioedema/chemically induced , Terminology as TopicABSTRACT
La enfermedad celíaca (EC) es una enfermedad autoinmune sistémica desencadenada por el consumo de gluten de la dieta en personas con susceptibilidad genética. Los principales test serológicos utilizados para el diagnóstico y seguimiento de la EC son pruebas basadas en anticuerpos de isotipo inmunoglobulina (Ig) A, siendo la determinación de IgA anti-transglutaminasa tisular (tTG)2 la prueba serológica inicial de elección. La deficiencia selectiva de IgA (DSIgA), es más prevalente en pacientes con EC que en la población general, dificultando el diagnostico serológico de la enfermedad. En el presente estudio observacional descriptivo, se incluyeron 74 pacientes adultos con diagnóstico confirmado de EC y se determinó IgA anti-tTG2 en suero mediante ensayo de ELISA a fin de detectar a aquellos pacientes con niveles indeterminados o negativos, los cuales podrían presentar DSIgA. Se dosó IgA total en el suero de estos pacientes por inmunodifusión radial y el promedio fue de 237,8 ± 100,6 mg/dL. En una paciente del sexo femenino fue detectada IgA total menor a 7 mg/dL, con niveles séricos de IgG e IgM normales, característicos de la DSIgA. Así, la frecuencia calculada de DSIgA fue de 1,35% en la población con EC estudiada. En conclusión, este trabajo es una primera aproximación para describir la frecuencia de DSIgA en pacientes con EC del país y reafirma la importancia de incluir el dosaje de IgA total en el caso de realizar test serológicos de la EC basados en IgA(AU)
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Immunoglobulin A/blood , Celiac Disease/blood , IgA Deficiency/blood , Celiac Disease/complications , Celiac Disease/immunology , Cross-Sectional Studies , IgA Deficiency/complications , IgA Deficiency/epidemiologyABSTRACT
INTRODUÇÃO: A deficiência de imunoglobulina A (DIgA) é a imunodeficiência primária mais comum e pode levar a quadros frequentes de infecções. Sua associação com lúpus eritematoso sistêmico (LES) é de extrema importância, dada a alta morbidade e mortalidade que as infecções causam nestes pacientes. OBJETIVOS: Demonstrar a prevalência da deficiência de IgA entre pacientes portadores de LES do sul do Brasil. Comparar o perfil clínico e de autoanticorpos entre pacientes lúpicos com e sem DIgA. PACIENTES E MÉTODOS: Estudo incluindo 189 pacientes com LES submetidos à dosagem sérica de IgA pelo método de nefelometria, sendo considerados deficientes aqueles com IgA inferior à 50 mg/dL. Dados demográficos, de perfil clínico [artrite, psicoses, convulsões, acidentes vasculares encefálicos (AVE), serosites, hemólise, leucopenia, plaquetopenia, nefrite] e de autoanticorpos [FAN, anti-SSA/Ro, anti-SSB/La, anti-Sm, anti-DNA, anti-RNP, LAC (anticoagulante lúpico) e aCL (anticorpos anticardiolipina)] IgG e IgM foram obtidos pela revisão de prontuários. Como controle, foram utilizados dados da literatura de um estudo feito na mesma área geográfica. Os dados foram analisados por tabelas de frequência e contingência aplicando-se os testes de Qui-quadrado, Fisher e Mann-Whitney. RESULTADOS: Foram encontrados 11 (6,17 por cento) pacientes com a DIgA (P < 0,001 em relação ao controle). O perfil clínico e de autoanticorpos dos pacientes com DIgA não foi diferente daquele dos pacientes sem essa deficiência. CONCLUSÃO: Pacientes com LES têm maior prevalência de DIgA que a população controle. A presença de DIgA em pacientes com LES não parece conferir qualquer particularidade clínica ou laboratorial aos mesmos.
INTRODUCTION: IgA deficiency (IgAD) is the most common primary immunodeficiency, which can cause frequent infections. The association of IgA deficiency with systemic lupus erythematosus (SLE) is very important because of the high morbidity and mortality rates of infections in patients with this disease. OBJECTIVES: To study the prevalence of IgA deficiency in SLE patients from southern Brazil and to compare the clinical and autoantibody profiles of SLE patients with and without IgA deficiency. PATIENTS AND METHODS: One hundred and eighty-nine SLE patients were submitted to serum IgA measurement by nephelometry. Levels of IgA below 50mg/dL were considered to be IgAD. Demographic data, clinical profile (presence of arthritis, psychosis, seizures, stroke, serositis, hemolytic anemia, leucopenia, thrombocytopenia, and nephritis) and autoantibody profiles (ANA, anti-Ro, anti-La, anti-Sm, anti-DNA, anti-RNP, lupus anticoagulant, and anticardiolipin IgG and IgM) were obtained from reviewing medical records. As control, we used literature data from another study performed in the same geographical area. Data were analyzed through contingency and frequency tables, applying the Chi-square, Fisher, and Mann Whitney tests. RESULTS: IgA deficiency was found in 11 (6.17 percent) patients (P < 0.001 in relation to controls). The association between IgA deficiency and clinical or autoantibody profile was not significant. CONCLUSION: We concluded that a higher prevalence of IgA deficiency was observed in lupus patients than in controls. Deficiency of IgA did not have any particular laboratory or clinical effects on this population.
Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Autoantibodies , IgA Deficiency/complications , IgA Deficiency/epidemiology , Lupus Erythematosus, Systemic/complications , IgA Deficiency/immunology , Lupus Erythematosus, Systemic/immunology , Prevalence , Retrospective StudiesABSTRACT
Wandering spleen is an uncommon clinical entity, which rarely affects children and adolescents. It is usually described in adults, being most common in the multiparous women of childbearing age. A case of a 14-year-old girl with a past history of splenomegaly and immunoglobulin A (IgA) deficiency, who presented with a sudden onset of abdominal pain, is presented. Diagnosis of hemoperitoneum secondary to torsion of a wandering spleen was made by computed tomography scan and Doppler ultrasound. Laparoscopy revealed hemoperitoneum owing to a ruptured and infarcted spleen. Laparotomy was undertaken and open splenectomy was successfully performed. The patient was discharged after an uneventful postoperative course that was not punctuated by any major complication. Management of this rare surgical emergency is discussed. Based on the details of this case, the authors hypothesize that IgA deficiency causes splenomegaly, which in turn predisposes to ligamentous laxity and splenic torsion.
Subject(s)
Adolescent , Diagnosis, Differential , Female , Hemoperitoneum/diagnosis , Humans , IgA Deficiency/complications , Tomography, X-Ray Computed , Ultrasonography, Doppler , Wandering Spleen/complicationsABSTRACT
OBJECTIVE: To characterize an IgA deficient and common variable immunodeficiency (CVI) group of patients in terms of the presence of rheumatologic manifestations. BACKGROUND: Although the molecular basis of some of the primary immunodeficiencies has been elucidated, it has not been possible to explain why in most cases these conditions are often associated with autoimmune manifestations, besides infections. The concomitant inability to fight infections adequately (immunodeficiency) and an inordinate reaction of the immune system to self components (autoimmunity) has been a perplexing situation. METHODS: The clinical and immunological profile of 71 patients fulfilling the diagnostic criteria of selective IgA deficiency (n=38) and common variable immunodeficiency (n=33) were evaluated for concurrent rheumatologic manifestations after a thorough medical history, physical examination and pertinent immunological parameters. RESULTS: The most common autoimmune conditions identified in patients with selective IgA deficiency were Crohn's disease and systemic lupus erythematosus (SLE); while immune thrombocytopenic purpura and Crohn's disease were the most common disorders associated to CVI. Anti-IgA antibodies were only found in 26.6(95C.I. 10.1-51.4) of patients with selective IgA deficiency but were present in all patients with that condition and SLE. Fifty per cent patients with CVI and ITP exhibited ANA positivity. CONCLUSIONS: The IgA-deficient group of patients in this study showed a higher prevalence of autoimmune conditions and greater positivity for ANA as compared to patients with CVI. In contrast to other reports with around 44positivity of anti-IgA antibodies in selective IgA patients these were only present in 263of patients with that disorder in this study. The high prevalence of antinuclear antibodies not associated with any clinical autoimmune condition in the IgA-deficient patients in this study will need to be further explored to ascertain why IgA-deficient patients may be at an increased risk of autoimmunity. Inflammatory bowel disease (Crohn's disease and ulcerative colitis) constituted the most common clinical autoimmune manifestations in both groups of patients studied. ITP was the commonest organ-specific autoimmune condition identified in the CVI group, as reported in previous publications. The limited number of patients studied does not allow a reliable estimate of the prevalence of SLE in the IgA-deficient population analyzed.
Subject(s)
Humans , Male , Female , Adolescent , Adult , IgA Deficiency/complications , Rheumatic Diseases/etiology , Common Variable Immunodeficiency/complications , Child , Child, Preschool , Hospitals, UniversityABSTRACT
Objetivos. Caracterizar os principais dados clínicos que orientem a pesquisa da DIgA e descrever sua evoluçao clínica e complicaçoes durante o seguimento ambulatorial. Material e Métodos. Foram avaliados, retrospectivamente, 60 pacientes portadores de DIgA (níveis séricos de IgA < 5 mg/dL) e submetidos à avaliaçao Clínica e provas laboratoriais específicas. Resultados. As queixas principais foram os processos infecciosos (50 por cento), quadros alérgicos (34 por cento) e doenças auto-imunes (10 por cento). Os processos infecciosos recorrentes acometeram preferencialmente o trato respiratório, sendo asma e rinite as manifestaçoes alérgicas mais frequentes. à avaliaçao laboratorial, além da deficiência de IgA, apenas os níveis de IgG e IgM encontravam-se elevados em 50 por cento dos pacientes. Durante a evoluçao, houve melhora da sintomatologia clínica e alguns pacientes tornaram-se assintomáticos durante a idade escolar e puberdade. Conclusao: A DIgA tem espectro clínico variado devendo ser sempre pesquisada em pacientes com infecçoes de repetiçao, processos alérgicos ou doenças auto-imunes para orientaçao de profilaxia precoce dos processos infecciosos e doenças alérgicas.
Subject(s)
Female , Humans , Infant , Child , Child, Preschool , Adolescent , Ambulatory Care , IgA Deficiency/diagnosis , Follow-Up Studies , IgA Deficiency/complications , Immunoglobulin G/blood , Immunoglobulin M/blood , Retrospective StudiesABSTRACT
Las inmunodeficiencias primarias constituyen entidades clínicas que, aun cuando se presentan esporádicamente en la práctica diaria, suelen acompañarse de gran morbilidad y mortalidad si no se efectúan un diagnóstico y un tratamiento oportunos. Algunas de estas inmunodeficiencias pueden iniciar su sintomatología con manifestaciones articulares que semejan un proceso reumatológico. De las numerosas inmunodeficiencias primarias descritas hasta la fecha, la hipogamaglobulinemia común variable es la entidad que con más frecuencia se asocia con estas manifestaciones. La enfermedad se caracteriza por una deficiencia severa en la producción de anticuerpos, lo que conduce a infecciones bacterianas recurrentes. En tanto, ciertas condiciones predisponentes para algunas enfermedades reumatológicas, tales como Lupus eritematoso Diseminado, Vasculitis, Nefropatía y otras. El conocimiento de estas condiciones inmunológicas reviste una gran importancia en la patogenia y manejo de las enfermedades reumatológicas