ABSTRACT
BACKGROUND: Acquired deficiency of C1 inhibitor (AAE-C1-INH) is a very rare cause of recurrent angioedema, with few cases reported in the literature. We aimed to describe a series of patients with AAE-C1-INH who were diagnosed and received care at angioedema reference centers in Brazil, affiliated to the Brazilian Group of Studies on Hereditary Angioedema. METHODS: Fourteen patients from 8 Brazilian Angioedema Reference Centers, diagnosed with AAE-C1-INH, were included in this study. Clinical data collected included sex, date of birth, date of onset of symptoms, date of diagnosis, plasma levels of antigenic and/or functional C1-INH, levels of C4 and C1q, location and treatment of angioedema attacks, long-term prophylaxis, associated diseases, and definitive treatment. RESULTS: Fourteen patients were identified with AAE-C1-INH. Most patients (10/14; 71.4%) were female. The median age at onset of symptoms was 56.5 years (range, 14-74 years; interquartile range [IQR], 32-64 years), and median age at diagnosis was 58.0 years (range, 20-76 years; IQR, 38-65 years), with a median time until diagnosis of 2 years (range, 0-6 years; IQR, 1-3 years). The most common manifestations were cutaneous (face, eyelids, lips, trunk, hands, feet, and genitals). Most patient had low levels of C4 (13/14; 92.8%) and of antigenic C1-INH (8/14; 57.1%). Four had decreased functional activity of C1-INH (4/7; 57.1%) and C1q levels were low in 5 patients (5/12; 41.6%). Underlying diseases were identified in all 14 patients, with lymphoma of the splenic marginal zone and monoclonal gammopathy of undetermined significance being the most frequent. Nine patients (64.2%) needed long-term prophylactic treatment for recurrent angioedema and 5 patients (46.7%) required treatment for angioedema attacks. Most of them (12/14; 85.7%) had resolution of angioedema. CONCLUSION: Therapy of AAE-C1-INH aims to control symptoms; however, diagnosis and treatment of the underlying disease, when present, should be an important target and may lead to the resolution of angioedema in patients with AAE-C1-INH.
Subject(s)
Angioedema , Angioedemas, Hereditary , Adolescent , Adult , Aged , Angioedema/diagnosis , Angioedema/etiology , Angioedemas, Hereditary/therapy , Brazil/epidemiology , Complement C1 Inhibitor Protein/genetics , Complement C1q/therapeutic use , Female , Humans , Male , Middle Aged , Young AdultABSTRACT
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.
Subject(s)
Anaphylaxis/diagnosis , Anaphylaxis/etiology , Drug Hypersensitivity/diagnosis , Drug Hypersensitivity/etiology , Perioperative Period , Adult , Allergy and Immunology , Anaphylaxis/physiopathology , Anesthesiology , Angioedema/chemically induced , Bradykinin/adverse effects , Brazil , Child , Drug Hypersensitivity/physiopathology , Humans , IgA Deficiency/complications , Immunoglobulin E/immunology , In Vitro Techniques , Mastocytosis/complications , Preoperative Care , Risk Factors , Skin Tests/methods , Societies, Medical , Symptom Assessment , Terminology as Topic , Vasodilator Agents/adverse effectsABSTRACT
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
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.
Subject(s)
Anesthesiology , Drug Hypersensitivity/etiology , Practice Guidelines as Topic , Anaphylaxis/etiology , Brazil , Humans , Perioperative PeriodABSTRACT
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
BACKGROUND: Asthma remains an uncontrolled disease. The Comorbidities, particularly obesity, and several other factors have been identified as being individually associated with asthma control, and these factors vary among different countries and between sexes. Studies have suggested that the harmful effects of these factors are greatest among women. The aim of the present study was to identify associated factors with uncontrolled asthma in women at the outpatient clinic of a Federal University Hospital in Rio de Janeiro, Brazil. METHODS: Cross-sectional study with asthmatic women, older than 18 years old. All subjects were included according to stringent criteria. The study used a structured questionnaire with data about demographic variables, education level, comorbid conditions, and disease history. Anthropometric and spirometric measurements were obtained. Asthma control was assessed by three different tools: the Asthma Control Test (ACT), the Asthma Control Questionnaire (ACQ) and the Global Initiative for Asthma (GINA) criteria. RESULTS: A total of 124 women were included, and 57%, 38% and 21% were totally controlled according to ACT, ACQ and GINA criteria, respectively. A total of 31.5% were obese. According to the CI (Conicity Index) and WC (waist circumference) respectively, 84% and 68% were centrally obese. There was no association between asthma control and age, education, duration of the disease or BMI (Body Mass Index) in the three models, but there was a significant association between central obesity and asthma control with the ACQ and GINA assessment tools, controlling for explanatory variables such as GERD (gastroesophageal reflux disease). Pre-bronchodilator percent predicted forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) were significantly associated with age and FVC was also associated with central obesity. CONCLUSIONS: Asthma remains uncontrolled in women despite treatment, and central obesity seems to have a negative influence on the control of the disease. We believe that women should be studied as a separate group and suggest prospective studies with assessment of fat distribution and biomarkers, controlling for possible comorbidities associated with asthma control.
