抽象的

Survey on hereditary angioedema in a German cohort

Hahn J*, Hoess A, Schuler PJ, Hoffmann TK, Mayer B, Greve J

Background and objectives: Hereditary angioedema (HAE) is a rare disease characterized by recurrent swelling attacks affecting almost every part of the body. So far, no reliable predictors for the clinical course have been identified. Here we evaluated the clinical situation of HAE patients in Germany and also aimed to analyze the role of body mass index (BMI) in disease severity.

Patients and methods: A survey among German HAE patients was done. The questions were related to the patients’ history, symptoms, and treatment. Groups with high and low BMI as well as different treatment modalities were compared.

Results: 61 patients were included. Fatigue proved the most frequent prodrome (37.7%), followed by psychiatric symptoms and skin changes. Swelling attacks predominantly affected the gastrointestinal tract, extremities and the head and neck region. About 60% of patients had at least monthly attacks. In most cases, first diagnosis was established by a dermatologist, followed by an ENT specialist and a general practitioner. Appropriate acute and prophylactic therapy reduced the number of days of sick leave by 81.3%. There was a tendency for a higher attack frequency in obese patients (BMI >30), but no statistically significant correlations could be established.

Conclusions: The majority of patients in this German cohort was provided state-of-the-art treatment, which lead to improved outcomes. Further studies assessing individual factors reflecting the disease severity like trigger factors or biomarkers may be considered.