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- Title
Hereditary Angioedema in Swedish Adults: Report From the National Cohort.
- Authors
NORDENFELT, Patrik; NILSSON, Mats; BJÖRKANDER, Janne; MALLBRIS, Lotus; LINDFORS, Anders; WAHLGREN, Carl-Fredrik
- Abstract
Hereditary angioedema (HAE) is rare, disabling and sometimes life-threatening. The aim of this study is to describe its prevalence, symptomatology and treatment in Sweden. A total of 146 patients were identified; 110 adults and 36 children with HAE type I (n = 136) or II (n = 10), giving a minimum HAE prevalence of 1.54/100,000. All patients received a written questionnaire followed by a structured telephone interview. This report focuses on the 102 adults who responded. Females reported 19 attacks in the previous year vs. 9 for males (p < 0.01), and females reported 10 days of sick leave vs. 4 days for males (p < 0.05). For all treated acute attacks, plasma-derived C1-inhibitor concentrate (pdC1INH) (used in 27% of patients) had a good effect. For maintenance treatment, 43% used attenuated androgens and 8% used pdC1INH, which reduced their attack rate by more than 50%. In conclusion, the minimum HAE prevalence in Sweden was 1.54/100,000. HAE affected females more severely. Attenuated androgens and pdC1INH had a good effect on preventing attacks.
- Subjects
ANGIONEUROTIC edema; SYMPTOMS; DISEASE prevalence; ANDROGENS; EPIDEMIOLOGY; GENETICS
- Publication
Acta Dermato-Venereologica, 2016, Vol 96, Issue 4, p540
- ISSN
0001-5555
- Publication type
Article
- DOI
10.2340/00015555-2274