Subjective assessment of sleep quality in adult patients with hereditary angioedema

Background: Hereditary angioedema (HAE) is a rare disorder characterized by recurrent swelling episodes, including painful abdominal attacks and life-threatening angioedema of the larynx that significantly affects patients' quality of life, including sleep. Sleep disorders have not yet been elucidated in HAE patients. Methods: This study evaluated sleep quality and insomnia by comparing attack-free periods with abdominal, head-neck, and extremity attacks. Sleep quality and insomnia were assessed using two validated questionnaires with the Basic Scale on Insomnia and Quality of Sleep (BaSIQS) and the Pittsburgh Sleep Quality Index (PSQI): sleep latency, difficulty falling asleep, night awakenings, problems returning to sleep, and overall sleep quality. Results: The study included 23 HAE patients; the median age was 31 years [interquartile range (IQR): 25-37], with a female predominance (n = 16, 69.5%). During the attack-free period, the median PSQI total score was 5 (IQR: 3.75 7.25), with 56.2% of the participants (n = 13) classified as good sleepers. Total PSQI scores significantly increased during attack periods compared to the attack-free period (median: 10 for abdomen, 8.5 for extremities, and 7.5 for head-neck; p < 0.001 for all). Whereas during the attack-free period, only 40.9% of patients exhibited good sleep quality (BaSIQS <9), with a median score of 10 (IQR: 7-14.2).Scores of BaSIQS significantly increased during angioedema attacks, with medians of 15 (extremity), 16 (abdominal), and 17 (head-neck), reflecting a notable decline in sleep quality. Among the components of PSQI, compared to the attack-free period, scores except the one assessing the need for medication to sleep all domains showed statistically significant increase. Conclusion: This study demonstrates that poor sleep quality, prolonged sleep latency, and increased awakenings are prevalent among HAE patients. Screening for sleep disorders and targeted interventions may help improve disease control and overall quality of life in HAE patients.