title: Quantifying the burden of disease and perceived health state in patients with hereditary angioedema in Sweden.
abstract: Hereditary angioedema (HAE) due to C1 inhibitor deficiency is a rare disease characterized by attacks of edema, known to impact quality of life (QoL). This study investigates the burden of HAE in Swedish patients, both children and adults. We used a retrospective registry study of Swedish patients with HAE, captured by the Sweha-Reg census. Data were collected using a paper-based survey. Patients completed EuroQoL 5 Dimensions 5 Levels (EQ5D-5L) questionnaires for both the attack-free state (EQ5D today), and the last HAE attack (EQ5D attack). Questions related to patient's age and sex and other variables, such as attack location and severity, were included to better understand the burden of HAE. EQ5D-5L values were estimated for the two HAE disease states. Patient-reported sick leave was also analyzed. A total of 103 responses were analyzed from 139 surveys (74% response rate). One hundred one reported an EQ5D today score (mean, 0.825) and 78 reported an EQ5D attack score (mean, 0.512) with significant differences between the two states (p < 0.0001). This difference was observed for both mild (p < 0.05), moderate (p < 0.0001), and severe attacks (p < 0.0001). Attack frequency had a negative effect on EQ5D today. Patients with >30 attacks a year had a significantly lower EQ5D today score than those with less frequent attacks. Of 74 participants, 33 (44.6%) had been absent from work or school during the latest attack and, of those with a severe attack, 81% had been absent. HAE has a significant impact on QoL both during and between attacks and on absenteeism during attacks.
authors.forename: Patrik
authors.initials: P
authors.lastname: Nordenfelt
authors.forename: Simon
authors.initials: S
authors.lastname: Dawson
authors.forename: Carl-Fredrik
authors.initials: CF
authors.lastname: Wahlgren
authors.forename: Anders
authors.initials: A
authors.lastname: Lindfors
authors.forename: Lotus
authors.initials: L
authors.lastname: Mallbris
authors.forename: Janne
authors.initials: J
authors.lastname: Björkander
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D054179:Angioedemas, Hereditary / Q000453:epidemiology* / Q000523:psychology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006304:Health Status; D006306:Health Surveys; D006801:Humans; D008297:Male; D008875:Middle Aged; D010465:Perception; D011788:Quality of Life; D012042:Registries; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D013548:Sweden / Q000453:epidemiology; D055815:Young Adult