Health-related quality of life and its risk factors in Chinese hereditary angioedema patients

Shuang Liu,Yingyang Xu,Xue Wang,Qun Xu,Yuxiang Zhi

doi:10.1186/s13023-019-1159-5

Abstract

BackgroundHereditary angioedema (HAE) is a rare but serious condition characterized by unpredictable and recurrent attacks affecting the skin and mucosa. HAE has wide-ranging impacts on the health-related quality of life (HRQoL) of patients. This study aims to assess the HRQoL of Chinese patients with HAE using the 36-item Short Form Health Survey (SF-36v2) and to explore potential risk factors for low HRQoL.MethodsA total of 104 patients (47 male and 57 female) over age 18 living in China with a known diagnosis of HAE due to C1-INH deficiency completed the SF-36v2 (generic HRQoL questionnaire). The results were compared to Chinese population norms. Subgroup analysis and logistic regression were used to interpret the data.ResultsSF-36v2 showed a significant reduction in all dimensions of HRQoL (p < 0.001) in patients with HAE compared with the general Chinese population. Female patients reported significantly lower bodily pain (BP) (p = 0.039) and physical component scores (PCSs) (p = 0.027) than male patients. Patients with mucosal edema tended to report lower role-physical (RP) limitations (p = 0.031) than patients with only skin edema. There were no differences between the mean scores of the SF-36 in relation to disease subtype, age, disease severity and long-term prophylaxis. Among female patients on long-term prophylaxis, social functioning (SF) (r = − 0.404, p = 0.010), role-emotional (RE) (r = − 0.320, p = 0.044) and mental component scores (MCSs) (r = − 0.313, p = 0.049) were negatively correlated with danazol dosage. A correlation between decreased disease control and decreased HRQoL scores was found, although the correlation was not significant in terms of RE or mental health (MH) scores. The logistic regression model revealed uncontrolled disease to be a risk factor for a low PCS (odds ratio 10.77, 95% confidence interval [CI] 1.78–65.06; p = 0.010) and laryngeal edema to be a risk factor for a low MCS (odds ratio 4.75, 95% CI 1.09–20.69; p = 0.038).ConclusionsChinese HAE patients reported significantly lower HRQoL scores than the general population. Unsatisfactory disease control is a risk factor for decreased PCSs. Laryngeal edema is a risk factor for decreased MCSs.

Highlights

Hereditary angioedema (HAE), a rare autosomal dominant disorder, is characterized by unpredictable and recurrent attacks of painful swelling that typically affect the extremities, bowel mucosa, genitals, face and upper airways [1]
Several forms of HAE have been defined: [1] type 1 HAE (HAE-1), which is caused by C1 inhibitor (C1-INH) deficiency and characterized by low C1-INH level and function; [2] type 2 HAE (HAE-2), which results from C1-INH dysfunction and is characterized by normal or slightly higher C1-INH levels but impaired function; and
The aim of this study was to assess the health-related quality of life (HRQoL) of Chinese patients with HAE using the 36-item Short Form Health Survey (SF-36v2) and to explore potential risk factors for decreased HRQoL

Summary

Introduction

Hereditary angioedema (HAE), a rare autosomal dominant disorder, is characterized by unpredictable and recurrent attacks of painful swelling that typically affect the extremities, bowel mucosa, genitals, face and upper airways [1]. The pathogenesis remains unknown in some HAE patients. The variability in disease manifestations and difficulties in differentiating HAE symptoms from those of more common angioedema subtypes account for a tendency of misdiagnosis and lead to a diagnostic delay typically longer than 10 years [4]. Hereditary angioedema (HAE) is a rare but serious condition characterized by unpredictable and recurrent attacks affecting the skin and mucosa. HAE has wide-ranging impacts on the health-related quality of life (HRQoL) of patients. This study aims to assess the HRQoL of Chinese patients with HAE using the 36-item Short Form Health Survey (SF-36v2) and to explore potential risk factors for low HRQoL

Objectives

Methods

Results