Determinants of health related quality of life (HRQoL) in adult patients with hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE)
Monday, March 5, 2018
South Hall A2 (Convention Center)
Marina Lluncor, M.D., Donatella Lamacchia, M.D., Adriana Hernanz, MS, Maria Pedrosa, M.D., Ph.D., Ana Alvez, M.D., Rosario Cabañas, M.D., Ph.D., Nieves Prior, M.D., Ph.D., Teresa Caballero, MD PhD

HRQoL is impaired in C1-INH-HAE, an inherited disease. We aimed to study HRQoL determinants in adult patients with C1-INH-HAE.


Hospital La Paz Ethics Committee approved the study (PI-2297). Spanish patients with C1-INH-HAE ≥18y were included. Demographic, clinical data were collected. HRQoL was measured by HAE-QoL, AE-QoL and EQ5D. A univariate statistical analysis was performed.


Fifty-six out of 61 patients were included (non-response rate:8.20%; mean age 46.7±14.0 y., 58.9% females).

Mean HAE-QoL score was 102.9±24.4, whereas mean adjusted AE-QoL score was 33.0±22.7 and mean EQ5D score was 0.86±0.17.

HRQoL (mean±SD) was more impaired in females than males [HAE-QoL (99.3±26.8 vs 107.8±20.3, n.s.), AE-QoL (37.7±23.5 vs 27.5±20.6, n.s.), EQ5D (0.82±0.20 vs 0.91±0.10, p=0.046)], in patients having had angioedema attacks in the last 6 months [HAE-QoL (98.40±23.58 vs 118.64±21.56,p<0.01), AE-QoL (16.52±18.27 vs 37.41±21.20,p<0.01); EQ5D (0.84±0.17 vs 0.93±0.08, n.s.)], in patients having had ≥ 6 versus 1-5 angioedema attacks in the last 6 months [HAE-QoL (87.71±22.92 vs 105.52±21.7,p=0.0262), AE-QoL (30.61±18.98 vs 47.37±20.89,p<0.01); EQ5D (0.78±0.23 vs 0.88±0.10, n.s.)] and in patients receiving long-term prophylaxis [HAE-QoL (91.3±25.4 vs 110.4±21.4,p<0.01), AE-QoL (40.9±23.6 vs 27.3±20.8,p=0.0298), EQ5D (0.76±0.22 vs 0.92±0.09,p<0.001)].

There were no significant differences in HRQoL regarding age, body max index, having family antecedents with C1-INH-HAE or having family antecedents of death due to asphyxia.


HRQoL in adult Spanish patients with C1-INH-HAE is lower in females, patients having had angioedema attacks in the last 6 months, those having had more than 6 angioedema attacks in the last 6 months and those receiving long term prophylaxis.