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