Hereditary Angioedema in Swedish Adults: Report From the National Cohort
2016 (English)In: Acta Dermato-Venereologica, ISSN 0001-5555, E-ISSN 1651-2057, Vol. 96, no 4, 540-545 p.Article in journal (Refereed) PublishedText
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 Cl-inhibitor concentrate (pdClINH) (used in 27% of patients) had a good effect. For maintenance treatment, 43% used attenuated androgens and 8% used pdClINH, 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 pdClINH had a good effect on preventing attacks.
Place, publisher, year, edition, pages
ACTA DERMATO-VENEREOLOGICA , 2016. Vol. 96, no 4, 540-545 p.
Clinhibitor deficiency; census; clinical manifestations; epidemiology; hereditary angioedema; prevalence; Sweden
IdentifiersURN: urn:nbn:se:liu:diva-128976DOI: 10.2340/00015555-2274ISI: 000375741300022PubMedID: 26540175OAI: oai:DiVA.org:liu-128976DiVA: diva2:934807
Funding Agencies|Futurum the Academy for Healthcare; Jonkoping County Council; Linkoping University; Karolinska Institutet2016-06-092016-06-072016-06-28