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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.

Bronchiectasis are common in Common Variable Immunodeficiency. These patients are prone to infection, leading to progressive lung destruction and accelerated FEV1 decline.40 year-old man, with recurrent respiratory infections, autoimmunity and diarrhea since age 7. At 17 CVID was diagnosed and IVIgG was started. During the following years, respiratory symptoms progressively worsened and bronchiectasis was found on thoracic computed tomography. Bronchoscopy revealed Pseudomonas aeruginosa in bronchoalveolar lavage and bronchial secretions cultures. Eradication therapy led to clinical improvement.This case report stresses the importance of regular microbiological screening and appropriate antibiotherapy. Early/aggressive treatment may significantly impact on patients' evolution.