Karabiber Esra, Güngören Ezgi Yalçın, Özen Ahmet, Barış Safa, Karakoc-Aydiner Elif
Department of Chest Disease, Division of Adult Allergy and Immunology, Pendik Training and Research Hospital, Istanbul, Türkiye.
Division of Pediatric Allergy and Immunology, Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Türkiye.
Front Neurol. 2025 Apr 16;16:1555562. doi: 10.3389/fneur.2025.1555562. eCollection 2025.
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.
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.
The study included 23 HAE patients; the median age was 31 years [interquartile range (IQR): 25-37], with a female predominance ( = 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 ( = 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; < 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.
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.
遗传性血管性水肿（HAE）是一种罕见的疾病，其特征为反复发作的肿胀，包括疼痛性腹部发作和危及生命的喉部血管性水肿，这会显著影响患者的生活质量，包括睡眠质量。HAE患者的睡眠障碍尚未得到阐明。
本研究通过比较无发作期与腹部、头颈和四肢发作期来评估睡眠质量和失眠情况。使用两份经过验证的问卷，即失眠与睡眠质量基本量表（BaSIQS）和匹兹堡睡眠质量指数（PSQI）来评估睡眠质量和失眠情况：睡眠潜伏期、入睡困难、夜间觉醒、再次入睡困难以及总体睡眠质量。
该研究纳入了23例HAE患者；中位年龄为31岁[四分位间距（IQR）：25 - 37]，女性占优势（n = 16，69.5%）。在无发作期，PSQI总分中位数为5（IQR：3.75 - 7.25），56.2%的参与者（n = 13）被归类为睡眠良好者。与无发作期相比，发作期PSQI总分显著升高（腹部发作期中位数为：10，四肢发作期为8.5，头颈发作期为7.5；所有均P < 0.001）。而在无发作期，只有40.9%的患者睡眠质量良好（BaSIQS < 9），中位数为10（IQR：7 - 14.2）。血管性水肿发作期间BaSIQS评分显著升高，四肢发作期、腹部发作期和头颈发作期的中位数分别为15、16和17，反映出睡眠质量明显下降。在PSQI的各项组成部分中，与无发作期相比，除评估睡眠所需药物这一项外，所有领域的评分均显示出统计学上的显著升高。
本研究表明，HAE患者中睡眠质量差、睡眠潜伏期延长和觉醒增加的情况普遍存在。筛查睡眠障碍并进行针对性干预可能有助于改善HAE患者的疾病控制和总体生活质量。
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