Molecular diagnosis and immunotherapy selection in a Portuguese population sensitized to grass and olive pollens / Diagnóstico molecular e seleção de imunoterapia numa população portuguesa sensibilizada ao pólen de gramíneas e de oliveira

Introduction: Phleum pratense (Phl p) and Olea europaea (Ole e) are common allergenic pollen. Objectives: To describe the sensitization patterns to Phl p and Ole e allergens in a subset of allergic rhinitis patients with positive skin prick tests (SPTs) to these pollens and compare the allergen immunotherapy (AIT) choice before and after determination of molecular components. Methods: Candidates to pollen immunotherapy with positive SPTs to both Phl p and Ole e were recruited. All of them underwent an SPT with a panel of aeroallergens and measurements of serum specific IgE (sIgE) to Phl p, Ole e, Phl p1, Phl p5, Phl p7, Phl p12, Ole e1, Ole e7, and Bet v2. Results: Forty adults were included. Of these, 83% and 65% were sIgE-positive to Phl p and Ole e, using the 0.35 kUA/L and 0.70 kUA/L cut-offs, respectively. Moreover, 42.5% of patients had positive sIgE to Phl p1 and/or Phl p5, 2.5% only to Ole e1, and 47.5% to both (0.35 kUA/L cutoff). By increasing the cut-off to 0.7 kUA/L, 55% of patients were sensitized to Phl p1 and/or Phl p5, and no patient was sensitized only to Ole e1. After component-resolved diagnosis, AIT choice was changed in 15 (37.5%) patients, with a decrease in the number of prescriptions of AIT with both grass and olive pollens and with olive alone, together with an increase in the prescriptions of AIT with grass pollen alone. Conclusion: Genuine sensitization to Olea europaea was reduced, and the sensitization patterns were heterogeneous. Knowledge of pattern of sensitization to molecular components changed immunotherapy prescription in more than one third of the patients.

Introdução: Os polens de Phleum pratense (Phl p) e de Olea europaea (Ole e) são fontes alergênicas comuns. Objetivos: Descrever os padrões de sensibilização aos alergênios destes dois polens num subconjunto de pacientes com rinite alérgica polínica e comparar a escolha de imunoterapia, antes e depois da determinação de alergênios moleculares para Phl p e Ole e. Métodos: Foram recrutados candidatos para imunoterapia com polens, com testes cutâneos positivos para Phl p e Ole e. Todos realizaram um painel de testes em picada a aeroalergênios e determinação de IgE séricas específicas para Phl p, Ole e, rPhl p1, rPhl p5, rPhl p7, rPhl p 12, rOle e 1, nOle e 7, rBet v2. Resultados: Foram incluídos 40 adultos. Em relação à sIgE para Phl p e Ole e, 83% e 65% dos pacientes apresentaram positividade para ambos, usando o cut-off de 0,35 kUA/L e 0,70 kUA/L, respectivamente. A positividade para Phl p1 e/ou Phl p 5 foi encontrada em 42,5%, para Ole e 1 apenas em 2,5%, enquanto 47,5% apresentaram sIgE positivo para ambos (cut-off corte de 0,35 kUA/L). Aumentando o cut-off para 0,7 kUA/L, 55% foram sensibilizados para Phl p1 e/ou Phl p5, nenhum paciente foi sensibilizado apenas para Ole e 1. Após a determinação dos alergênios para os componentes moleculares, a escolha de imunoterapia foi alterada em 15 (37,5%) pacientes, com uma diminuição no número de vacinas para Phleum + Olea e apenas para Olea e um aumento na prescrição de vacinas para Phleum. Conclusão: A sensibilização genuína do Olea europaea foi reduzida e os padrões de sensibilização foram heterogêneos. O conhecimento da sensibilização aos componentes moleculares dos alergênios mudou a prescrição de imunoterapia em mais de um terço dos pacientes.

Hospitalizations due to Angioedema without Urticaria in a Portuguese Center: Five Year Retrospective Study.

INTRODUCTION: Hospitalizations due to angioedema are important especially in debilitating or life-threatening situations. The aim of this study was to evaluate the frequency and etiology of angioedema without urticaria in hospital admissions. MATERIAL AND METHODS: The admissions between 2009 and 2013 in Centro Hospitalar Lisboa Norte with a diagnosis grouped under the ICD9 codes of angioedema were retrospectively analysed. The episodes of angioedema with urticaria were excluded. The admissions were categorized into 2 groups: A - hospitalizations motivated by the angioedema; B - hospitalizations in which the angioedema was an incidental finding. RESULTS: There were 169 hospitalizations (52% females, 96% adults, mean age 52 ± 20.8 years), distributed by 23 hospital departments, 51% in the Immunoallergology department. The mean annual angioedema admission rate was 72/100 000. In 68% of the cases, angioedema was the cause for the admission; in 32% an incidental finding. In 38% there was upper airway involvement. The etiologies were: hereditary angioedema in 24%, angiotensin converting enzyme inhibitor induced angioedema in 31%, idiopathic angioedema in 21%, thrombolysis induced angioedema in 13%, nonsteroidal anti-inflammatory drug-induced angioedema in 5%. DISCUSSION: The main etiology was angiotensin converting enzyme inhibitor angioedema, followed by hereditary angioedema and thrombolysis induced angioedema, and these findings concur with the international literature. CONCLUSION: The mean annual angioedema admission rate was 72/100 000 and there was airway involvement in 38% of hospitalizations.

Introdução: Os internamentos por angioedema são importantes, sobretudo, nas situações incapacitantes ou de risco de vida. O objetivo deste estudo foi avaliar a frequência e etiologia dos internamentos por angioedema sem urticária. Material e Métodos: Estudo retrospetivo dos internamentos com os códigos CID9 para angioedema, entre 2009 e 2013, no Centro Hospitalar Lisboa Norte. Foram excluídos os episódios de angioedema com urticária. Categorizaram-se os internamentos em dois grupos: A ­ Internamentos motivados pela crise de angioedema; B ­ Internamentos em que o angioedema foi uma intercorrência. Resultados: Foram incluídos 169 internamentos (53% mulheres, 96% adultos, idade média 52 ± 20,8 anos), distribuídos por 23 serviços hospitalares (51% na Imunoalergologia), com uma taxa média anual de internamentos de 72/ 100 000. Em 68% o angioedema foi o motivo de internamento, em 32% uma intercorrência. Em 38% houve envolvimento das vias aéreas superiores. As etiologias foram: em 24% angioedema hereditário, em 31% angioedema induzido por inibidores da enzima conversora da angiotensina, 21% foram idiopáticos, em 13% o angioedema surgiu após trombólise, em 5% induzido por anti-inflamatórios não esteroides e 7% outras etiologias. Discussão: A principal etiologia foi o angioedema induzido por inibidores da enzima conversora da angiotensina, seguido do angioedema hereditario e angioedema associado a trombolise, sendo estes dados semelhantes a outras revisões internacionais. Conclusão: A taxa média de internamentos por angioedema foi de 72/100 000 e a frequência de envolvimento das vias aéreas foi de 38%.

Expert perspectives on hereditary angioedema: Key areas for advancements in care across the patient journey.

BACKGROUND: Published literature documents the substantial burden of hereditary angioedema (HAE) with C1 inhibitor deficiency on the quality of life and work productivity of patients. However, despite advances in the field and the availability of guidelines to advise health care providers (HCP) on the diagnosis and management of HAE, there are still many challenges to overcome. For example, delayed diagnosis and misdiagnosis are common, and treatment practices vary worldwide. OBJECTIVE: An international expert panel was convened to consider opportunities for improvements that would benefit patients with HAE. METHODS: Based on professional and personal experiences, the experts developed schematics to describe the journey of patients through the following stages: (1) onset of symptoms and initial evaluation; (2) referral/diagnosis; and (3) management of HAE. More importantly, the panel identified key areas in which it was possible to optimize the support provided to patients and HCPs along this journey. RESULTS: Overall, this approach highlighted the need for wider dissemination of algorithms and scientific data to more effectively educate HCPs from multiple disciplines and the need for more research to inform appropriate treatment decisions. Furthermore, HAE awareness campaigns, accurate online information, and referral to patient advocacy groups were all considered helpful approaches to support patients. CONCLUSION: More detailed and widespread information on the diagnosis and management of HAE is needed and may lead to advancements in care throughout the journey of the patient with HAE.

[Approach and Registry of Anaphylaxis in Portugal]. / Abordagem e Registo da Anafilaxia em Portugal.

Anaphylaxis has a growing incidence, especially in children. It represents a medical emergency and its successful therapy depends on early and proper intervention. Intramuscular epinephrine, with dose adjustment according to weight and age, is the drug of choice for anaphylaxis treatment. After resolution of the acute reaction, the patient should be kept under clinical surveillance for 6 to 24 hours, due to possible biphasic reactions. Prescription of an epinephrine auto injectable device should be considered in all patients with diagnosed or suspected anaphylaxis; additionally these patients should always be referred to an Immunoallergy consultation, to perform adequate investigation and management in order to reduce future risk. All anaphylaxis episodes must be recorded in The Portuguese Catalogue of Allergies and other Adverse Reactions (Catálogo Português de Alergias e outras Reações Adversas, CPARA), which represents a fundamental tool to share clinical information within the Health System. The present manuscript intends to disclose the most recent Portuguese guidelines for the diagnosis and treatment of anaphylaxis, making its clinical approach more effective and homogeneous, and to promote the use of The Portuguese Catalogue of Allergies and other Adverse Reactions as an essential tool to register and share information on anaphylaxis in Portugal.

A anafilaxia apresenta uma incidência crescente, particularmente em idade pediátrica. Constituindo uma emergência Médica, o sucesso terapêutico depende de uma intervenção precoce e adequada. A adrenalina por via intramuscular constitui o fármaco de eleição para o seu tratamento, devendo a dose ser ajustada ao peso e à idade. Resolvida a reação aguda, o doente deve ser mantido sob vigil'ncia Médica por um período de 6 a 24 horas, pelo risco de ocorrência de reações bifásicas. Deverá ser considerada a prescrição de um dispositivo de autoadministração de adrenalina em todos os doentes com diagnóstico ou suspeita de anafilaxia; adicionalmente estes doentes têm indicação formal para estudo em consulta de imunoalergologia, de modo a permitir uma adequada intervenção diagnóstica e terapêutica que reduzirá o risco futuro. Todos os episódios de anafilaxia devem ser registados no Catálogo Português de Alergias e outras Reações Adversas (CPARA), constituindo este um instrumento fundamental de partilha de informação clínica dentro do Sistema de Saúde. Este manuscrito pretende divulgar as orientações para o diagnóstico e tratamento da anafilaxia, tornando a sua abordagem clínica mais eficiente e consertada a nível nacional, e promover a adesão ao Catálogo Português de Alergias e outras Reações Adversas como um instrumento essencial de registo e partilha de informação dos episódios de anafilaxia ocorridos em Portugal.

Are general practitioners alert to anaphylaxis diagnosis and treatment?

INTRODUCTION: Anaphylaxis is a potentially lethal condition. Prevention of mortality depends on the correct diagnosis and on early epinephrine injection by the patients affected. OBJECTIVE: Evaluate practical knowledge of diagnostic and therapeutic aspects of anaphylaxis in general practitioners. MATERIAL AND METHODS: 100 GPs were inquired through the use of an anonymous and voluntary questionnaire with 5 multiple-choice questions (just one correct answer): 3 about diagnostic aspects and two about therapeutic aspects of anaphylaxis. There was also one other question where it was asked the commercial name of the only device for epinephrine auto-injection available in Portugal. Eight minutes were given to fulfil the questionnaire. 92 complete questionnaires were eligible for evaluation. RESULTS: In the three diagnostic questions we observed a percent of correct answers ranging from 36 to 46%. In the two questions regarding anaphylaxis therapy only 18 to 22% of the GPs gave the correct answers. Only 14% of the GPs were able to indicate the correct commercial name of the epinephrine auto-administration kit. CONCLUSIONS: The results of this study stress the need to promote, within the GP community, continuous medical education programmes regarding adequate diagnosis and treatment of anaphylaxis. Wrong concepts related to anaphylaxis treatment are frequently observed and this is a serious problem since GPs are the front-line doctors in the management of out-patient anaphylactic reactions and therefore it is absolutely mandatory that they can be able not only to accurately diagnose and treat an acute anaphylactic episode but also to make a correct retrospective diagnosis and to advise on the use of epinephrine auto-injectors.