Omalizumab in Chronic Spontaneous Urticaria: A Brazilian Real-Life Experience.

BACKGROUND: Current guidelines on chronic spontaneous urticaria (CSU) suggest a treatment based on a 3-step approach that aims at total symptom control, starting with H1-antihistamines. However, a significant number of patients present an antihistamine-resistant urticaria that must be treated with an alternative third-line therapy such as omalizumab. METHODS: Patients with a history of CSU who did not respond to treatment with high doses of modern antihistamines were treated with 150 or 300 mg of omalizumab every 4 weeks. The response to treatment was recorded as complete (CR), partial (PR) or no response. A dose adjustment was proposed according to response. RESULTS: We treated 47 CSU patients with omalizumab (40 females), of whom 39.5% had evidence of autoimmunity. The average number of treatments was 11.4 (range 2-87). All patients had been refractory to high-dose modern antihistamines. A CR was seen in 84.6% of patients who started with 300 mg and in 60% of those who started with 150 mg. Only 1 patient had no response to both the 150- and 300-mg doses. In 6 of the PR patients with 150 mg, a higher dose of 300 mg was proposed and 4 had a CR. Four patients discontinued the treatment. No severe adverse events were reported in the patients who finished the study. DISCUSSION: Although good results were seen in both groups, CR rates were higher in those under a high-dose initial treatment. Our data strongly suggest that the therapy should be individualized.

Angioedema hereditário com deficiência do Inibidor de C1 ­ Armadilhas no diagnóstico, tratamento e compreensão / Hereditary angioedema with C1 inhibitor deficiency: traps in the diagnosis, treatment, and understanding

Angioedema hereditário (AEH) é uma condição rara, subdiagnosticada e de elevada morbimortalidade, devido ao caráter de suas manifestações clínicas. O AEH se diferencia do angioedema histaminérgico por não responder aos anti-histamínicos, corticosteroides ou epinefrina. Por esse motivo, é extremamente importante o diagnóstico dessa situação, a fim de instituir a terapia adequada. Tal afecção deve ser suspeitada a partir da história clínica de episódios imprevisíveis e recorrentes de edema que quando se manifesta sob a forma de edema laríngeo, pode levar a óbito por asfixia, se não for adequadamente tratado. Relatamos o caso de uma paciente de 18 anos que, apesar de previamente diagnosticada com AEH tipo 1, ao procurar um serviço de emergência devido a crise de angioedema, não dispunha de medicação específica nem apresentou plano de ação com as opções possíveis para crises. Este caso reforça a necessidade de maior divulgação da doença, além da conscientização de pacientes e familiares sobre a doença e eventuais crises, assim como o acesso as medicações.

Hereditary angioedema (HAE) is a rare, underdiagnosed condition with high morbidity and mortality due to the characteristics of its clinical presentation. HAE differs from histaminergic angioedema by not responding to antihistamines, corticosteroids, or epinephrine. Therefore, early diagnose is crucial to initiate adequate therapy. HAE is suspected in patients with a clinical history of unpredictable and recurrent episodes of edema. When laryngeal edema occurs, it can lead to death from asphyxiation if not treated properly. We report the case of an 18-year-old patient previously diagnosed with HAE type 1 who sought emergency care during an angioedema attack. However, the patient was not taking any specific medication and did not have an action plan to manage attacks. This case highlights the importance of increasing awareness about the disease, educating patients and their families about the disease and potential attacks, and ensuring access to medications.

Allergic bronchopulmonary aspergillosis' diagnosis remains a challenge.

INTRODUCTION: Allergic bronchopulmonary aspergillosis (ABPA) is a complex disease, triggered by a hypersensitivity reaction to the allergens of Aspergillus fumigatus, a fungus that opportunistically colonizes the lungs of patients with asthma. The diagnosis of ABPA is difficult. A major problem is the lack of standardized allergens used in the determination of specific IgE, but the use of recombinant allergens has been proposed to overcome this. The aim of the present study is to evaluate whether serological tests for IgE specific to recombinant allergens of A. fumigatus (rAsp) can aid in the detection of sensitization to this fungus and in the diagnosis of ABPA. METHODS: This was an observational, cross-sectional study. The diagnosis of ABPA, using classical criteria, was searched in 65 asthmatics patients with immediate cutaneous reactivity to A. fumigatus. After that, serum titers of IgE against rAsp f 1, rAsp f 2, rAsp f 3, rAsp f 4 and rAsp f 6 were determined. In order to compare the differences between patients with confirmed and excluded diagnosis of ABPA, the two-tailed Fisher's exact test was used. RESULTS: Although 19 of 65 patients had IgE against at least one recombinant, the disease was diagnosed in only six patients by classical criteria. One of them had IgE against all recombinant allergens tested and another one had antibody against Asp f 3. DISCUSSION: The determination of serum IgE against recombinant A. fumigatus allergens in this group was not helpful to make the diagnosis of ABPA, neither to detect sensitization to fungus.

Hereditary angioedema and Allergic bronchopulmonary aspergillosis: an unexpected association / Angioedema hereditário e Aspergilose broncopulmonar alérgica: uma associação inesperada

Hereditary angioedema (HAE) is a rare autosomal dominant disorder, Allergic bronchopulmonary aspergillosis (ABPA) is a lung disease involving hypersensitivity to the fungi Aspergillus fumigatus which occur in susceptible patient with asthma or cystic fibrosis, also considered a rare disease. We report a case of HAE and ABPA in a single patient. HAE diagnosis was confirmed: C4 = 3 mg/dL, C1INH < 2.8 mg/dL - nephelometry. Former lung function showed elevation RV and RV/FVC, suggesting small airways lung disease. Positive skin prick test to Aspergillus fumigatus (03 mm); total serum IgE level 3,100 IU/mL (nephelometry - BNII Siemens), eosinophilia 11% (528/mm3) and specific A. fumigatus IgG antibodies 6,8 mgA/L (FEIA - fluorenzymeimmunoassay - ThermoFisher) and Chest CT showed mucoid impaction of the bronchi, consistent to current ABPA. Controlling ABPA could prevent and reduce angioedema attacks, and lung structural damage. Early diagnosis and treatment of both diseases should be emphasized to reduce mortality and morbidity

Angioedema hereditário (AEH) é uma doença autossômica dominante; aspergilose broncopulmonar alérgica (ABPA) é uma doença de hipersensibilidade pulmonar relacionada ao esporo de Aspergillus fumigatus, mais suscetível em pacientes com asma e fibrose cística, ambas são consideradas doenças raras. Apresentamos um caso de AEH e ABPA em um paciente. O diagnóstico de AEH foi confirmado com exames laboratoriais: C4 = 3 mg/dL, C1INH < 2,8 mg/dL - nefelometria. Prova de função pulmonar evidenciou aumento de VR e VR/CVF, sugerindo doenças de pequenas vias aéreas. Teste de puntura positivo para A. fumigatus (03 mm); IgE total = 3.100 IU/mL (nefelometria - BNII Siemens), eosinofilia 11% (528/mm3) e IgG específica para A. fumigatus 6,8 mgA/L (FEIA - ThermoFisher), TC de tórax evidenciou impactação mucoide, consistente com ABPA. Controlar ABPA pode prevenir e reduzir as crises de angioedema e os danos ao tecido pulmonar. O diagnóstico precoce de ambas as doenças deve ser enfatizado para reduzir a morbimortalidade.

Testes de provocação para urticárias crônicas induzidas: a experiência de um centro de referência e excelência em urticária - UCARE / Provocation tests for chronic inducible urticaria: the experience of a urticaria center of reference and excellence

Introdução: A urticária é determinada pela ativação de mastócitos que se apresenta por urticas, angioedema ou ambos. A urticária é classificada de acordo quanto a sua duração, em duas formas: aguda (UA < 6 semanas) e crônica (UC > 6 semanas). A UC compreende Urticária Crônica Espontânea (UCE) e Urticárias Crônicas Induzidas (UCInd). Entre as UCInd estão o dermografismo, urticária por pressão tardia (UPT), frio, calor, solar, aquagênica, colinérgica e urticária/angioedema vibratório. As UCInd podem ser diagnosticadas por meio da história clínica, exame físico e da reprodução das lesões através dos testes de provocação. Objetivo: Descrever o perfil dos testes de provocação positivos para UCInd realizados em um Centro de Referência e Excelência em Urticária (GA2LEN UCARE). Métodos: Foram avaliados, retrospectivamente, os resultados dos testes de provocação para UCInd, realizados de dezembro de 2017 a setembro de 2021, de 114 pacientes que apresentavam história sugestiva de uma ou mais UCInd. Resultados: Dos 114 pacientes avaliados, oitenta e oito (77%) eram do sexo feminino e 26 (23%) do masculino. Foram diagnosticados, através de testes de provocação positivos: 65 dermografismos (FricTest® e/ou dermografômetro); 23 UPT (23 diagnosticados com o uso do dermografômetro e 11 também confirmados através do teste de Warin); 11 urticárias ao frio (temperaturas iguais ou inferiores a 27 °C) e 3 urticárias ao calor (temperaturas iguais ou superiores a 38 °C), todos diagnosticados com o TempTest® versão 4.0; 4 urticárias colinérgicas, diagnosticados através do Teste Modificado para Urticária Colinérgica - HUCFF-UFRJ e 1 urticária vibratória. Nenhum paciente apresentou teste positivo para urticária solar ou aquagênica. Sete pacientes foram negativos. Conclusão: Os testes de provocação, através do estímulo direto e seguro com o desencadeante, permitem ao médico avaliador e ao paciente a compreensão e a confirmação do estímulo causador da enfermidade em questão e seus limiares.

Introduction: Urticaria is determined by mast cell activation that presents as wheals, angioedema, or both. Urticaria is classified according to its duration into two forms: acute (< 6 weeks) and chronic (> 6 weeks). Chronic urticaria includes chronic spontaneous urticaria and chronic inducible urticaria. Chronic inducible urticarias include dermographism, delayed pressure urticaria, cold, heat, solar, aquagenic, cholinergic, and vibratory urticaria/angioedema. Chronic inducible urticaria can be diagnosed through clinical history, physical examination, and the reproduction of lesions through provocation tests. Objective: To describe the profile of positive provocation tests for chronic inducible urticaria performed at an urticaria center of reference and excellence (GA2LEN UCARE). Methods: We retrospectively evaluated the results of provocation tests performed between December 2017 and September 2021 in 114 patients with a history suggestive of one or more types of chronic inducible urticaria. Results: The sample included 88 (77%) female and 26 (23%) male patients. The following were diagnosed through positive provocation tests: 65 cases of dermographism (FricTest® and/or dermographometer); 23 cases of delayed pressure urticaria (all diagnosed with a dermographometer and 11 confirmed with the Warin test); 11 cases of cold urticaria (temperatures ≤ 27°C) and 3 cases of heat urticaria (temperatures ≥ 38°C), all diagnosed with TempTest® 4.0; 4 cases of cholinergic urticaria, all diagnosed with the Modified Test for Cholinergic Urticaria-HUCFFUFRJ, and 1 case of vibratory urticaria. No patient tested positive for solar or aquagenic urticaria. Seven patients have been negative. Conclusion: Provocation tests, which use direct and safe stimuli as triggers, allow physicians and patients to confirm the disease's causative stimulus and its thresholds.

Guia prático de urticária para grupos especiais de pacientes / Practical guide to urticaria for special patient groups

A urticária crônica é uma condição que afeta mais de um milhão de brasileiros, com grande impacto na qualidade de vida. Mesmo com diretrizes bem difundidas para o seu diagnóstico e tratamento, seu manejo pode ser desafiador em pacientes pediátricos, idosos e gestantes. Para auxiliar o médico especialista nestes casos, o Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia elaborou esta revisão com as principais dúvidas e dificuldades referentes ao tema nestes grupos de pacientes.

Chronic urticaria is a condition that affects more than a million Brazilians with a significant impact on quality of life. Although there are well-established guidelines for diagnosis and treatment, the management of chronic urticaria may be challenging in pediatric, older, and pregnant patients. With the purpose of helping specialists manage these cases, the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology prepared this review with the most common doubts and difficulties about this topic in those patient groups.

Guia prático de urticária aguda / Practical guide to acute urticaria

A urticária aguda é uma causa frequente de consulta com alergistas, caracterizada por urticas e/ou angioedema. Embora autolimitada e benigna, pode causar desconforto significativo e raramente representar uma doença sistêmica grave ou reação alérgica com risco de vida. Nesta revisão, elaborada pelo Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia, foram abordadas as principais questões referentes ao tema para auxiliar o médico especialista e generalista.

Acute urticaria is a frequent cause of consultations with allergists, being characterized by wheals and/or angioedema. Although self-limited and benign, it may cause significant discomfort and uncommonly represent a serious systemic disease or life-threatening allergic reaction. In this review prepared by the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology, the main questions about this topic are addressed to help specialists and general practitioners.

Staphylococcal superantigen-specific IgE antibodies: degree of sensitization and association with severity of asthma.

OBJECTIVE:: To determine the presence of staphylococcal superantigen-specific IgE antibodies and degree of IgE-mediated sensitization, as well as whether or not those are associated with the severity of asthma in adult patients. METHODS:: This was a cross-sectional study involving outpatients with asthma under treatment at a tertiary care university hospital in the city of Rio de Janeiro, Brazil. Consecutive patients were divided into two groups according to the severity of asthma based on the Global Initiative for Asthma criteria: mild asthma (MA), comprising patients with mild intermittent or persistent asthma; and moderate or severe asthma (MSA). We determined the serum levels of staphylococcal toxin-specific IgE antibodies, comparing the results and performing a statistical analysis. RESULTS:: The study included 142 patients: 72 in the MA group (median age = 46 years; 59 females) and 70 in the MSA group (median age = 56 years; 60 females). In the sample as a whole, 62 patients (43.7%) presented positive results for staphylococcal toxin-specific IgE antibodies: staphylococcal enterotoxin A (SEA), in 29 (20.4%); SEB, in 35 (24.6%); SEC, in 33 (23.2%); and toxic shock syndrome toxin (TSST), in 45 (31.7%). The mean serum levels of IgE antibodies to SEA, SEB, SEC, and TSST were 0.96 U/L, 1.09 U/L, 1.21 U/L, and 1.18 U/L, respectively. There were no statistically significant differences between the two groups in terms of the qualitative or quantitative results. CONCLUSIONS:: Serum IgE antibodies to SEA, SEB, SEC, and TSST were detected in 43.7% of the patients in our sample. However, neither the qualitative nor quantitative results showed a statistically significant association with the clinical severity of asthma. OBJETIVO:: Determinar a presença de anticorpos IgE específicos para superantígenos estafilocócicos e o grau de sensibilização mediada por esses, assim como se esses estão associados à gravidade da asma em pacientes adultos. MÉTODOS:: Estudo transversal incluindo asmáticos adultos em acompanhamento ambulatorial em um hospital universitário terciário no Rio de Janeiro (RJ). Os pacientes foram alocados consecutivamente em dois grupos de gravidade da asma segundo critérios da Global Initiative for Asthma: asma leve (AL), com asmáticos leves intermitentes ou persistentes, e asma moderada ou grave (AMG). Foram determinados os níveis séricos de anticorpos IgE antitoxinas estafilocócicas, e os resultados foram comparados por análise estatística. RESULTADOS:: Foram incluídos 142 pacientes no estudo: 72 no grupo AL (mediana de idade = 46 anos; 59 do sexo feminino) e 70 do grupo AMG (mediana de idade = 56 anos; 60 do sexo feminino). Na amostra geral, 62 pacientes (43,7%) apresentaram resultados positivos para dosagens de anticorpos IgE antitoxinas estafilocócicas: enterotoxina (TX) A, em 29 (20,4%); TXB, em 35 (24,6%); TXC, em 33 (23,2%); e toxic shock syndrome toxin (TSST), em 45 (31,7%). As médias das dosagens séricas de anticorpos IgE específicos anti-TXA, TXB, TXC e TSST foram, respectivamente, de 0,96 U/l, 1,09 U/l, 1,21 U/l, e 1,18 U/l. Não houve diferença estatisticamente significativa dos resultados qualitativos ou quantitativos entre os grupos. CONCLUSÕES:: A presença de anticorpos IgE séricos anti-TXA, TXB, TXC e TSST, foi detectada em 43,7% nessa amostra de pacientes, mas não houve associação estatisticamente significativa entre seus resultados qualitativos ou quantitativos e gravidade clínica da asma.

Impact of chronic urticaria on the quality of life of patients followed up at a university hospital.

BACKGROUND:: Chronic urticaria is a debilitating disease that considerably affects health-related quality of life, and the Chronic Urticaria Quality of Life Questionnaire is the only questionnaire specifically designed for its evaluation. OBJECTIVE:: To evaluate the quality of life of patients with chronic urticaria, using the Brazilian Portuguese version of the Chronic Urticaria Quality of Life Questionnaire. METHODS:: The Chronic Urticaria Quality of Life Questionnaire was self-administered in 112 chronic urticaria patients and disease activity was assessed through the Urticaria Activity Score. Clinical and socio-demographic characteristics of patients were studied, such as: age, sex, etiologic diagnosis of chronic urticaria, duration of disease and Urticaria Activity Score. RESULTS:: The population studied was composed 85.72% of women with a mean age of 46 years (18-90), while the median disease duration period was 10 years (3 months-60 years). Regarding the etiologic diagnosis, 48.22% had chronic spontaneous urticaria; 22.32% associated with inducible urticaria, 28.57% with chronic autoimmune urticaria, and 23.21% had physical urticaria alone. Disease activity evaluated using the Urticaria Activity Score was 1.04 ± 1.61 (0-6). The total score for the Chronic Urticaria Quality of Life Questionnaire was 36 (0-100) and dimension I (sleep/mental status/eating) had a greater impact on quality of life. The items with the highest mean scores were nervousness and shame over lesions, while the items with the lowest scores were lip swelling and limitations on sporting activities. CONCLUSIONS:: Chronic urticaria compromises patients' quality of life, mainly those with more severe disease or who are diagnosed with chronic autoimmune urticaria.

Vacina COVID-19 em pacientes com urticária ­ Posicionamento do Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia (ASBAI) / COVID-19 vaccine in patients with urticaria ­ Position statement of the Scientific Department of Urticaria of the Brazilian Association of Allergy and Immunology (ASBAI)

Com o início do programa de vacinação contra a COVID-19 no Brasil, surgiu uma série de questionamentos relacionados ao uso dos imunizantes em pacientes com doenças imunoalérgicas. Neste documento, o Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia (ASBAI) se posiciona revisando as principais dúvidas relacionadas à imunização para COVID-19 em pacientes com urticária.

As the COVID-19 vaccination program started in Brazil, many questions have arisen regarding the use of vaccines in patients with immune-allergic diseases. In this document, the Scientific Department of Urticaria of the Brazilian Association of Allergy and Immunology takes a stand by reviewing the main queries regarding COVID-19 immunization in patients with urticaria.

Urticária e a COVID-19 / Urticaria and COVID-19

A pandemia de COVID-19 afetou drasticamente a vida de todos ao redor do planeta, interferindo também na forma de atuarmos como médicos e especialistas. Neste artigo revisamos aspectos importantes da infecção pelo novo coronavírus e sua relação com a urticária.

The COVID-19 pandemic has dramatically affected people's lives around the world and has interfered with how we act as physicians and specialists. In this paper, we review important aspects of the new coronavirus infection and its connection with urticaria.

Urticárias crônicas induzidas: atualização do tema / Chronic inducible urticaria: topic update

A urticária é uma doença comum, determinada pela ativação de mastócitos que se apresenta por urticas, angioedema, ou ambos. Convencionou-se classificar a urticária, quanto a sua duração, em duas formas: aguda (UA) e crônica (UC). A urticária é definida como crônica quando persiste por 6 semanas ou mais. A urticária crônica compreende urticária crônica espontânea (UCE) e urticárias crônicas induzidas (UCInd), que incluem as urticárias físicas e não físicas. Estudos sugerem que a presença de UCInd associada a UCE está ligada a um pior prognóstico e duração da doença. Essa revisão tem por objetivo atualizar as informações disponíveis sobre a prevalência, quadros clínicos, métodos diagnósticos e tratamentos das UCInd por estímulos físicos ou não.

Urticaria is a common disease determined by the activation of mast cells that presents with urticaria, angioedema, or both. According to its duration, urticaria is classified into two forms: acute (AU) and chronic (CU). Urticaria is defined as CU when it persists for 6 weeks or more. CU consists of chronic spontaneous urticaria (CSU) and chronic inducible urticaria (CIndU), which includes physical and nonphysical urticarias. Studies suggest that the presence of both CIndU and CSU is linked to worse prognosis and longer duration of these diseases. This review aims to update available information on the prevalence, clinical manifestations, diagnostic methods, and treatments of CIndU by physical or nonphysical stimuli.

Guia prático do tratamento com omalizumabe para urticária crônica espontânea / Practical guide to omalizumab treatment for chronic spontaneous urticaria

A urticária crônica é uma doença com grande impacto socioeconômico e na qualidade de vida do indivíduo. O adequado conhecimento de formas de tratamento eficazes e com perfil de segurança satisfatório, assim como dos mecanismos preditores de resposta ao tratamento, são essenciais para que se alcance um controle adequado da doença. O omalizumabe é um anticorpo monoclonal anti-IgE com eficácia reconhecida e bom perfil de segurança no tratamento da urticária crônica. Objetivamos elucidar questões envolvidas no manejo desta medicação, através da revisão em literatura de estudos atuais e com relevância clínica. Foi realizado levantamento de questões importantes e pouco elucidadas, buscando respostas baseadas nestes estudos. Com isso, foram abordados aspectos práticos do tratamento com o omalizumabe, esclarecendo desde os fenótipos dos pacientes e conduta adequada para estas diferentes situações, trazendo possíveis fatores preditores de resposta ao tratamento e contemplando também um novo anticorpo monoclonal anti-IgE no manejo destes pacientes.

Chronic urticaria is a disease with great socioeconomic impact on people's quality of life. Adequate knowledge of effective treatments with a satisfactory safety profile as well as of the predictive mechanisms of response to treatment are essential for achieving adequate disease control. Omalizumab is an anti-IgE monoclonal antibody with recognized efficacy and a good safety profile in the treatment of chronic urticaria. We aim to elucidate issues involving the management of this medication through a literature review of current studies with clinical relevance. A survey of important and unclear questions was conducted, and the answers were sought in the studies. Thus, practical aspects of omalizumab treatment were addressed, including the phenotypes of patients and appropriate approaches for different situations. Possible predictive factors of response to treatment and a new anti-IgE monoclonal antibody for the management of these patients were also reported.

Icatibant, an inhibitor of bradykinin receptor 2, for hereditary angioedema attacks: prospective experimental single-cohort study.

CONTEXT AND OBJECTIVE: Hereditary angioedema (HAE) with C1 inhibitor deficiency manifests as recurrent episodes of edema involving the skin, upper respiratory tract and gastrointestinal tract. It can be lethal due to asphyxia. The aim here was to evaluate the response to therapy for these attacks using icatibant, an inhibitor of the bradykinin receptor, which was recently introduced into Brazil. DESIGN AND SETTING: Prospective experimental single-cohort study on the efficacy and safety of icatibant for HAE patients. METHODS: Patients with a confirmed HAE diagnosis were enrolled according to symptoms and regardless of the time since onset of the attack. Icatibant was administered in accordance with the protocol that has been approved in Brazil. Symptom severity was assessed continuously and adverse events were monitored. RESULTS: 24 attacks in 20 HAE patients were treated (female/male 19:1; 19-55 years; median 29 years of age). The symptoms were: subcutaneous edema (22/24); abdominal pain (15/24) and upper airway obstruction (10/24). The time taken until onset of relief was: 5-10 minutes (5/24; 20.8%); 10-20 (5/24; 20.8%); 20-30 (8/24; 33.4%); 30-60 (5/24; 20.8%); and 2 hours (1/24; 4.3%). The time taken for complete resolution of symptoms ranged from 4.3 to 33.4 hours. Adverse effects were only reported at injection sites. Mild to moderate erythema and/or feelings of burning were reported by 15/24 patients, itching by 3 and no adverse effects in 6. CONCLUSION: HAE type I patients who received icatibant responded promptly; most achieved improved symptom severity within 30 minutes. Local adverse events occurred in 75% of the patients.

Ferramentas para avaliação e acompanhamento da urticária crônica / Patient-reported outcomes for the evaluation and follow-up of chronic urticaria

Urticária é uma doença pruriginosa da pele na qual ocorrem urticas e/ou angioedema. A urticária é definida como crônica quando persiste por 6 semanas ou mais. A urticária crônica tem um grande impacto na vida diária do paciente. Atualmente, não há biomarcadores confiáveis para identificar e medir a atividade da doença na urticária crônica espontânea. Consequentemente, o uso de ferramentas conhecidas por patient-reported outcomes (PROs) é crucial ao avaliar e monitorar diferentes aspectos da urticária crônica, como atividade/gravidade da doença, controle da doença e qualidade de vida. Apresentamos uma visão geral de cinco PROs usados na avaliação da urticária crônica, e destacamos suas vantagens, limitações e uso na prática clínica e pesquisa.

Urticaria is an itching skin disease characterized by the presence of wheals and/or angioedema. Urticaria is defined as chronic when it persists for 6 weeks or more. Chronic urticaria has great impact on the daily lives of patients. Currently, there are no reliable biomarkers to identify and measure disease activity in chronic spontaneous urticaria. Consequently, the use of tools known as patient-reported outcomes (PROs) is crucial when evaluating and monitoring different aspects of chronic urticaria such as disease activity/severity, disease control, and quality of life. We present an overview of the five PROs used in the evaluation of chronic urticaria, highlighting their advantages, limitations, and use in clinical practice and research.

Avaliação do teste do soro autólogo e do teste do plasma autólogo na urticária crônica espontânea / Evaluation of autologous serum skin test and autologous plasma skin test in chronic spontaneous urticaria

Objetivo: Comparar o teste do soro autólogo (TSA) e o teste do plasma autólogo (TPA) para o diagnóstico de autorreatividade em pacientes com urticária crônica espontânea (UCE) e avaliar sua relação com autoanticorpos antitireoidianos. Métodos: Cinquenta pacientes com UCE foram pareados por sexo e faixa etária a 50 indivíduos saudáveis (controle) e comparados em relação aos resultados do TSA e TPA e autoimunidade tireoidiana. Resultados: Vinte e três (46%) pacientes e 5 (10%) controles apresentaram TSA positivo; 16 (32%) pacientes e 7 (14%) controle apresentaram TPA positivo. Houve associação entre função tireoidiana e TSA em pacientes com UCE. Pacientes eutireóideos com UCE tiveram taxa maior de TSA positivo. Conclusão: O TSA é simples e barato e contribui consideravelmente para a elucidação da patogênese da UCE e pode demonstrar a presença de autoanticorpos funcionais. Alguns autores sugerem que o TPA é mais sensível que o TSA. Todos os estudos relevantes sobre o assunto até o momento reportaram que ambos podem ser utilizados como métodos diagnósticos em pacientes com UCE. Concluindo, TSA e TPA podem ser utilizados para a avaliação de autorreatividade na etiologia da UCE, e autoanticorpos antitireoidianos devem ser investigados mesmo quando testes de função tireoidiana revelam-se normais.

Objective: To compare the autologous serum skin test (ASST) and the autologous plasma skin test (APST) for the diagnosis of autoimmunity in patients with chronic spontaneous urticaria (CSU) and to evaluate their relationship with antithyroid autoantibodies. Methods: Fifty patients with CSU and 50 sex- and age-matched healthy controls were compared in relation to ASST and APST results and thyroid autoimmunity. Results: Twenty-three (46%) patients and 5 (10%) controls had positive ASST; 16 (32%) patients and 7 (14%) controls had positive APST. There was an association between thyroid function and ASST results in patients with CSU. Euthyroid patients with CSU had a higher rate of positive ASST. Conclusion: ASST is a simple, cost-effective test that significantly contributes to elucidate the pathogenesis of CSU and can demonstrate the presence of functional autoantibodies. Some authors suggest that APST is more sensitive than ASST. All relevant studies published so far report that both can be used as diagnostic methods in patients with CSU. In conclusion, ASST and APST can both be used to evaluate autoreactivity in the etiology of CSU, and antithyroid autoantibodies should be investigated even when thyroid function tests are normal.

Atopy patch test (APT) in the diagnosis of food allergy in children with atopic dermatitis.

BACKGROUND: Atopic Dermatitis is a chronic inflammatory skin disease. Food allergens are important in the pathogenesis in 1/3 of the cases. Several mechanisms are involved in the pathogenesis of Atopic Dermatitis. Immediate reactions are identified by both measurement of specific IgE and skin prick test. Atopy Patch Test seems to be relevant in the investigation of patients with suspected delayed-type reactions. OBJECTIVES: To evaluate the standardization of this method concerning allergen concentration, occlusion time and interpretation, and determine the specificity and sensitivity of the Atopy Patch Test according to the skin prick test and specific IgE levels in food allergy diagnosis in children with Atopic Dermatitis. METHODS: Seventy-two children, aged 2-12 years were selected and followed at the allergy clinic of the Hospital São Zacharias. Skin prick test, specific IgE and food Atopy Patch Test (cow's milk, egg, soy and wheat) were carried out. Three groups were submitted to the Atopy Patch Test: (1) Atopic Dermatitis with or without Rhinitis and Asthma; (2) Rhinitis and or Asthma without AD; (3) Healthy individuals. RESULTS: In group 1, 40% of the patients presented positive reactions. The longer the exposure time (48h and 72h), the higher the sensitivity. In group 2, the test was more specific than sensitive for all the extracts, with increased sensitivity the longer the time of exposure (72h). In group 3, 8.3% presented positive tests. CONCLUSION: APT evidenced a great diagnostic value in late-phase reactions to food, with high specificity. It showed to be a specific and reliable tool in comparison with the healthy group's results.

Prevalence of autoantibodies in a group of hereditary angioedema patients.

Hereditary Angioedema is a dominantly inherited disease. Routine screening of autoantibodies (AAB) is not recommended for individuals with Hereditary Angioedema; however, prevalence of these antibodies in Hereditary Angioedema patients is not well documented. We aim to determine the prevalence of AAB so that individuals at risk of developing autoimmune diseases can be identified. Fifteen patients with Hereditary Angioedema attended at Clementino Fraga Filho University Hospital accepted to participate in this study. Prevalence of AAB was 40%. Our data indicate high prevalence of AAB in patients with Hereditary Angioedema. Large-scale studies should be considered to determine the significance of these AAB in the follow-up care of patients with Hereditary Angioedema.