title: Hereditary Angioedema in Swedish Adults: Report From the National Cohort.
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 minimal 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 minimal 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.
authors.affiliation: Department of Internal medicine, Pulmonary medicine and allergology, , 551 85 Jönkoping, Sweden. patrik.nordenfelt@telia.com.
authors.forename: Patrik
authors.initials: P
authors.lastname: Nordenfelt
authors.forename: Mats
authors.initials: M
authors.lastname: Nilsson
authors.forename: Janne
authors.initials: J
authors.lastname: Björkander
authors.forename: Lotus
authors.initials: L
authors.lastname: Mallbris
authors.forename: Anders
authors.initials: A
authors.lastname: Lindfors
authors.forename: Carl-Fredrik
authors.initials: CF
authors.lastname: Wahlgren
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000728:Androgens / Q000627:therapeutic use; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D005260:Female; D019538:Health Care Surveys; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D012074:Remission Induction; D012189:Retrospective Studies; D018582:Sick Leave; D013548:Sweden / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000728:Androgens; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents; C469952:SERPING1 protein, human