Hereditary angioedema (HAE) is a syndrome characterizedby episodic swelling of subcutaneous and submucosal tissues, due to a deficiency or dysfunctionality in functionalC1 esterase inhibitor (C1-inh). The authors present a caseof a 40 year-old man with a positive family history of HAE,recurrent episodes of abdominal pain, nausea and vomiting.The immunological study revealed low levels of C1-inh andC4 and normal levels of C1q. Acquired angioedema wasexcluded, making the diagnosis of type 1 HAE. The patientpresented several times to the emergency department withsevere abdominal pain, diffuse abdominal tenderness withguarding. However, laboratory and imagiologic studies hadno alterations. A subcutaneous injection of icatibant was administered with resolution of symptoms. HAE is a rare andpotentially fatal disease, mainly during acute attacks. An early diagnosis and a proper treatment are fundamental.

O angioedema hereditário (AEH) é uma síndrome caracterizada por episódios recorrentes de edema submucoso e/ou subcutâneo, associados à deficiência quantitativa ou funcional do inibidor C1-esterase (C1-inib). Os autores apresentam o caso de um homem, de 40 anos, com história familiar de AEH. Apresentava crises recorrentes de dor abdominal, náuseas e vómitos. O estudo imunológico revelou níveis diminuídos de C1-inib e C4 e os de C1q eram normais. Excluiu-se angioedema adquirido, diagnosticando-se AEH tipo 1. Recorreu várias vezes ao serviço de urgência por dor abdominal intensa, com abdómen distendido, difusamente doloroso. Contudo o estudo analítico e imagiológico não apresentavam alterações. Foi administrada injecção subcutânea de icatibant, com resolução do quadro. O AEH é uma doença rara, potencialmente fatal, nomedamente durante as crises. É fundamental o diagnóstico atempado e terapêutica adequada.

RESUMO Fundamento: Medir o controlo da doença alérgica é fundamental na avaliação dos resultados da imunoterapia com alergénios (ITA). Objetivo: Descrever o controlo da doença alérgica (asma, rinite, conjuntivite) em doentes sob ITA subcutânea (ITSC). Métodos: Estudo descritivo, incluindo todos os indivíduos com >12 anos sob ITSC entre 03/2017 e 06/2019 nas unidades de Imunoalergologia da CUF‑Porto que responderam a ≥1 questionário de avaliação prévia à administração de ITSC em uso nas unidades; analisou‑se apenas o questionário mais recente de cada indivíduo. O questionário incluiu uma escala visual analógica (EVA) de sintomas oculares (0, mínimo, a 10 cm, máximo) e o CARAT. O controlo da asma e rinite foi definido como CARAT total (CARATT)>24; o controlo das vias aéreas superiores (VAS) e inferiores (VAI) baseou‑se nas pontuações parciais (CARATVAS>8 e CARATVAI≥16, respetivamente). Resultados: Incluiram‑se 209 indivíduos, 96 (46%) do sexo feminino e 62 (30%) com <18 anos. Todos tinham rinite e 32% (n=67) tinham asma. 34% estavam no primeiro ano da ITSC e 40% no terceiro ou mais. A pontuação média (desvio-padrão) do CARATT foi de 24,6(4,2), com 57% (n=119) dos indivíduos controlados. 75% (n=50) dos doentes com asma apresentavam controlo das VAI, mas apenas 47% (n=98) tinham a rinite controlada. As pontuações do CARATT e CARATVAS não se correlacionaram significativamente com a duração da ITSC (r=0,082 e - 0,011, respetivamente). A pontuação do CARATVAI apresentou uma correlação significativa positiva com a duração do tratamento (r=0,203). A pontuação mediana (intervalo interquartil) da EVA de sintomas oculares foi de 0,3(0,8) cm; 79% tiveram pontuação <1 cm. Conclusões: Cerca de 60% dos doentes apresentaram controlo da doença alérgica respiratória, mas apenas 47% tinham a rinite controlada. Apesar da proporção de controlo ser superior às publicadas na literatura para doentes alérgicos, são necessários outros estudos de vida real para reforçar a evidência de efetividade da imunoterapia com alergénios.

Background Previous studies have demonstrated the feasibility of using an asthma app to support medication management and adherence but failed to compare with other measures currently used in clinical practice. However, in a clinical setting, any additional adherence measurement must be evaluated in the context of both the patient and physician perspectives so that it can also help improve the process of shared decision making. Thus, we aimed to compare different measures of adherence to asthma control inhalers in clinical practice, namely through an app, patient self-report and physician assessment. Methods This study is a secondary analysis of three prospective multicentre observational studies with patients (≥13 years old) with persistent asthma recruited from 61 primary and secondary care centres in Portugal. Patients were invited to use the InspirerMundi app and register their inhaled medication. Adherence was measured by the app as the number of doses taken divided by the number of doses scheduled each day and two time points were considered for analysis: 1-week and 1-month. At baseline, patients and physicians independently assessed adherence to asthma control inhalers during the previous week using a Visual Analogue Scale (VAS 0–100). Results A total of 193 patients (72% female; median [P25–P75] age 28 [19–41] years old) were included in the analysis. Adherence measured by the app was lower (1 week: 31 [0–71]%; 1 month: 18 [0–48]%) than patient self-report (80 [60–95]) and physician assessment (82 [51–94]) (p < 0.001). A negligible non-significant correlation was found between the app and subjective measurements (ρ 0.118–0.156, p > 0.05). There was a moderate correlation between patient self-report and physician assessment (ρ = 0.596, p < 0.001). Conclusions Adherence measured by the app was lower than that reported by the patient or the physician. This was expected as objective measurements are commonly lower than subjective evaluations, which tend to overestimate adherence. Nevertheless, the low adherence measured by the app may also be influenced by the use of the app itself and this needs to be considered in future studies.

Background: Health and fitness apps have potential benefits to improve self-management and disease control among patients with asthma. However, inconsistent use rates have been reported across studies, regions, and health systems. A better understanding of the characteristics of users and nonusers is critical to design solutions that are effectively integrated in patients’ daily lives, and to ensure that these equitably reach out to different groups of patients, thus improving rather than entrenching health inequities. Objective: This study aimed to evaluate the use of general health and fitness apps by patients with asthma and to identify determinants of usage. Methods: A secondary analysis of the INSPIRERS observational studies was conducted using data from face-to-face visits. Patients with a diagnosis of asthma were included between November 2017 and August 2020. Individual-level data were collected, including age, gender, marital status, educational level, health status, presence of anxiety and depression, postcode, socioeconomic level, digital literacy, use of health services, and use of health and fitness apps. Multivariate logistic regression was used to model the probability of being a health and fitness app user. Statistical analysis was performed in R. Results: A total of 526 patients attended a face-to-face visit in the 49 recruiting centers and 514 had complete data. Most participants were ≤40 years old (66.4%), had at least 10 years of education (57.4%), and were in the 3 higher quintiles of the socioeconomic deprivation index (70.1%). The majority reported an overall good health status (visual analogue scale [VAS] score>70 in 93.1%) and the prevalence of anxiety and depression was 34.3% and 11.9%, respectively. The proportion of participants who reported using health and fitness mobile apps was 41.1% (n=211). Multivariate models revealed that single individuals and those with more than 10 years of education are more likely to use health and fitness mobile apps (adjusted odds ratio [aOR] 2.22, 95%CI 1.05-4.75 and aOR 1.95, 95%CI 1.12-3.45, respectively). Higher digital literacy scores were also associated with higher odds of being a user of health and fitness apps, with participants in the second, third, and fourth quartiles reporting aORs of 6.74 (95%CI 2.90-17.40), 10.30 (95%CI 4.28-27.56), and 11.52 (95%CI 4.78-30.87), respectively. Participants with depression symptoms had lower odds of using health and fitness apps (aOR 0.32, 95%CI 0.12-0.83). Conclusions: A better understanding of the barriers and enhancers of app use among patients with lower education, lower digital literacy, or depressive symptoms is key to design tailored interventions to ensure a sustained and equitable use of these technologies. Future studies should also assess users’ general health-seeking behavior and their interest and concerns specifically about digital tools. These factors may impact both initial engagement and sustained use.

Health and fitness apps have potential benefits to improve self-management and disease control among patients with asthma. However, inconsistent use rates have been reported across studies, regions, and health systems. A better understanding of the characteristics of users and nonusers is critical to design solutions that are effectively integrated in patients’ daily lives, and to ensure that these equitably reach out to different groups of patients, thus improving rather than entrenching health inequities.

 Anxiety and depression are relevant comorbidities in asthma, but, in Portugal and Spain, data on this topic are scarce. We assessed, in patients with asthma, the frequency of anxiety and depression using the Hospital Anxiety and Depression Scale (HADS) and the European Quality of Life Five Dimension Questionnaire (EQ-5D); the level of agreement between these questionnaires, and the factors associated with these symptoms. This is a secondary analysis of the INSPIRERS studies. A total of 614 adolescents and adults with persistent asthma (32.6±16.9 years, 64.7% female) were recruited from 30 primary care centres and 32 allergy, pulmonology and paediatric clinics. Demographic and clinical characteristics, HADS and EQ-5D were collected. A score ≥8 on Hospital Anxiety and Depression Scale-Anxiety/Hospital Anxiety and Depression Scale-Depression or a positive answer to EQ-5D item 5 indicated the presence of these symptoms. Agreement was determined by Cohen’s kappa. Two multivariable logistic regressions were built. According to HADS, 36% of the participants had symptoms of anxiety and 12% of depression. According to EQ-5D, 36% of the participants had anxiety/depression. The agreement between questionnaires in identifying anxiety/depression was moderate (k=0.55, 95% CI 0.48 to 0.62). Late asthma diagnosis, comorbidities and female gender were predictors of anxiety/depression, while better asthma control, health-related quality of life and perception of health were associated with lower odds for anxiety/depression. At least 1/3 of the patients with persistent asthma experience symptoms of anxiety/depression, showing the relevance of screening these disorders in patients with asthma. EQ-5D and HADS questionnaires showed a moderate agreement in the identification of anxiety/depression symptoms. The identified associated factors need to be further investigated in long-term studies.

A iniciativa Allergic Rhinitis and Its Impact on Asthma (ARIA) teve início há mais de 20 anos e tem elaborado e disseminado orientações baseadas em evidência, e desenvolvido projetos na área da rinite alérgica. Esta iniciativa está atualmente focada em proporcionar orientações centradas no doente que contribuam para um percurso integrado entre os vários níveis de cuidados e que tirem partido de soluções digitais, tendo sido recomendada a introdução na prática clínica de percursos assistenciais integrados. Neste artigo descrevemos a adaptação para Portugal do documento ARIA Integrated Care Pathways. Após breve revisão sobre a epidemiologia e o impacto da rinite alérgica em Portugal e das atividades realizadas em Portugal no âmbito da iniciativa ARIA, é descrito o conjunto alargado de conhecimento utilizado para o desenvolvimento das recomendações para o tratamento farmacológico da rinite alérgica, recomendações essas baseadas na metodologia GRADE, evidência do mundo real adquirida por tecnologia móvel (mHealth) e resultante de estudos de câmara de exposição alergénica. Em seguida, são resumidos os percursos assistenciais integrados para imunoterapia com alergénios produzidas em 2019. Considera-se a imunoterapia com alergénios um exemplo de medicina de precisão e em que a utilização de tecnologias mHealth permitirá melhorar a estratificação para seleção dos doentes e monitorização da resposta. Estas recomendações foram consideradas como ‘boas práticas’ dos cuidados integrados centrados no doente apoiados por sistemas digitais da DG Santé (Direção Geral de Saúde e de Segurança Alimentar da União Europeia) e representam a estratégia de gestão da mudança da fase 4 do ARIA.