Hereditary angioedema: 24 years of experience in a Portuguese Reference Center.

Summary: Hereditary angioedema (HAE) poses a high burden of disease, being its epidemiological and clinical data heterogeneous among countries, with no recent published studies concerning Portuguese patients. Therefore, we aimed to raise awareness of HAE and to contribute to clinical knowledge. An observational, descriptive, retrospective, and cross-sectional study was performed, that included a cohort of 126 patients followed in a single Portuguese Center. We observed a high prevalence of HAE-C1-INH type II (45.2% of patients). Most HAE patients (67.4%) presented the initial manifestations of the disease before adulthood, at a mean age of 12.6 ± 8.4 years. However, we found a long delay in HAE diagnosis, especially in those without family history (mean 20.7 ± 17.3 years). Stress was the most common trigger, followed by trauma and infection. Symptoms involving different systems were increasingly reported with increased disease duration. Cutaneous symptoms (95.0%) were more frequent, followed by gastrointestinal (80.7%), and respiratory symptoms (50.4%). HAE symptoms led to abdominal surgery in 22 (17.5%) patients and induced laryngeal edema requiring intubation/tracheostomy in 8 (6.3%) patients. Most patients were under long-term prophylaxis, mainly with attenuated androgens (62.7% of patients).The correct distinction between HAE and other common causes of angioedema is critical, allowing reduction of diagnostic delay, improvement of adequate management, and ultimately improving outcomes and quality of life of HAE patients.

Lupine sensitisation in a population of 1,160 subjects.

Lupin is part of the Mediterranean diet and is also used as a thickener of food products. It has been recognised as a cause of serious allergic reactions. This study aims at determining the prevalence of lupin sensitisation in 1,160 subjects consulting allergologists. This population performed skin prick tests (SPT) to lupin. In case of positivity, a clinical questionnaire was applied and the subjects were tested for other legumes, common inhalants and latex. We find a 4.1% sensitisation rate to lupin, a 75% co-sensitisation between lupin and legumes, a 82.1% co-sensitisation between lupin and pollen and a 28.5% co-sensitisation between lupin and latex. To conclude, we documented a high lupin sensitisation in a selected population, thus stressing the importance of lupin as a food allergen.