Nordenfelt Patrik, Nilsson Mats, Lindfors Anders, Wahlgren Carl-Fredrik, Björkander Janne
Allergy Asthma Proc. 2017 Nov 30;38(6):447-455. doi: 10.2500/aap.2017.38.4087. Epub 2017 Aug 30.
Health-related quality of life (HR-QoL) is impaired in patients with hereditary angioedema (HAE) but has not yet been satisfactorily described.
To study HR-QoL in patients with HAE by combining different HR-QoL instruments with disease activity assessment.
All adults in the Swedish HAE registry were invited to take part in this questionnaire study, which used the generic HR-QoL instruments, EuroQol 5 Dimensions 5 Level (EQ-5D-5L) and the RAND Corporation Short Form 36 (RAND-36), the disease-specific Angioedema Quality of Life instrument (AE-QoL), the recently introduced Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication.
Sixty-four of 133 adults (26 men, 38 women) between 18 and 91 years old responded. The most affected HR-QoL dimensions in the EQ-5D-5L were pain/discomfort and anxiety/depression; in the RAND-36, energy/fatigue, general health, pain; and, in the AE-QoL, fears/shame and fatigue/mood. Women had lower HR-QoL in the RAND-36 for general health and energy/fatigue (p < 0.05). Patients who reported any AAS of >0 had significantly impaired HR-QoL. There were significant associations (p < 0.05) between the AAS and EQ-5D-5L, between the AAS and all dimensions of the RAND-36 except physical function, and between the AAS and AE-QoL in all dimensions. Nine of 36 patients who reported sick leave during the previous 4 weeks had significantly impaired HR-QoL in all the instruments (p < 0.05). There was no significant difference in HR-QoL in the patients with and the patients without prophylactic medication, except for the nutrition dimension of the AE-QoL (p < 0.05).
Comprehensive information is obtained by combining different HR-QoL instruments. Pain, anxiety/depression, and fatigue/mood are important aspects of HAE but the AE-QoL disregards pain. HR-QoL was not significantly affected by prophylaxis. Increased disease activity was associated with impaired HR-QoL, which justifies more active disease management.
遗传性血管性水肿（HAE）患者的健康相关生活质量（HR-QoL）受损，但尚未得到令人满意的描述。
通过将不同的HR-QoL工具与疾病活动评估相结合，研究HAE患者的HR-QoL。
邀请瑞典HAE登记处的所有成年人参与这项问卷调查研究，该研究使用了通用的HR-QoL工具，即欧洲五维健康量表5级（EQ-5D-5L）和兰德公司36项简短健康调查（RAND-36）、疾病特异性血管性水肿生活质量工具（AE-QoL）、最近推出的血管性水肿活动评分（AAS）表，以及关于病假和预防性药物治疗的问卷。
133名年龄在18至91岁之间的成年人（26名男性，38名女性）中有64人做出了回应。EQ-5D-5L中受影响最大的HR-QoL维度是疼痛/不适和焦虑/抑郁；在RAND-36中是精力/疲劳、总体健康、疼痛；在AE-QoL中是恐惧/羞耻和疲劳/情绪。女性在RAND-36中的总体健康和精力/疲劳方面的HR-QoL较低（p<0.05）。报告任何AAS>0的患者的HR-QoL显著受损。AAS与EQ-5D-5L之间、AAS与RAND-36除身体功能外的所有维度之间以及AAS与AE-QoL的所有维度之间均存在显著相关性（p<0.05）。在过去4周内报告病假的36名患者中有9名在所有工具中的HR-QoL均显著受损（p<0.05）。使用和未使用预防性药物治疗的患者在HR-QoL方面没有显著差异，但AE-QoL的营养维度除外（p<0.05）。
通过结合不同的HR-QoL工具可获得全面信息。疼痛、焦虑/抑郁和疲劳/情绪是HAE的重要方面，但AE-QoL忽略了疼痛。预防性治疗对HR-QoL没有显著影响。疾病活动增加与HR-QoL受损相关，这证明需要更积极地管理疾病。
