Merlo Christian A, Yin Linda X, Hoag Jeffrey B, Mitchell Sally E, Reh Douglas D
Department of Medicine and Epidemiology, Johns Hopkins Medicine, Baltimore, MD.
Int Forum Allergy Rhinol. 2014 Nov;4(11):921-5. doi: 10.1002/alr.21374. Epub 2014 Aug 21.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease mainly characterized by epistaxis in more than 96% of patients. Recently, a validated questionnaire known as the HHT Epistaxis Severity Score (ESS) was developed. However, little is known about the relationship between epistaxis and quality of life. We hypothesize that epistaxis severity is a major factor predicting health-related quality of life (HR-QoL) in HHT patients.
This is a cross-sectional study. The ESS questionnaire and Medical Outcomes Study 36-item short form (SF-36) were administered to subjects through an Internet survey. All participants had a definitive diagnosis of HHT through Curaçao criteria or genetic testing. Demographic information, genetics, and extensive histories were also collected. Descriptive analyses were performed with calculations of means and standard deviations (SDs) for continuous variables and proportions for categorical variables. Linear regressions were then performed to assess the association between HR-QoL and ESS.
A total of 604 subjects participated between April and August 2008. All patients reported epistaxis, 285 (47.2%) had telangiectasias, and 545 (90.2%) had a family history of HHT; 167 (27.6%) patients had mild epistaxis (ESS <4), 285 (47.2%) reported moderate epistaxis (≥4 ESS <7), and 152 (25.2%) reported severe epistaxis (ESS ≥7). Patients with severe epistaxis had lower scores for both the Physical Component Summary (PCS) and the Mental Component Summary (MCS) of HR-QoL when compared to those with mild epistaxis (p < 0.001, p < 0.001).
The ESS is a major determinant of HR-QoL and should be considered as a measurement of treatment efficacy in HHT-related epistaxis.
遗传性出血性毛细血管扩张症（HHT）是一种常染色体显性疾病，96%以上的患者主要表现为鼻出血。最近，一种经过验证的问卷，即HHT鼻出血严重程度评分（ESS）被开发出来。然而，关于鼻出血与生活质量之间的关系知之甚少。我们假设鼻出血严重程度是预测HHT患者健康相关生活质量（HR-QoL）的主要因素。
这是一项横断面研究。通过互联网调查向受试者发放ESS问卷和医学结局研究36项简表（SF-36）。所有参与者均通过库拉索标准或基因检测确诊为HHT。还收集了人口统计学信息、遗传学信息和详细病史。对连续变量计算均值和标准差（SDs），对分类变量计算比例，进行描述性分析。然后进行线性回归，以评估HR-QoL与ESS之间的关联。
2008年4月至8月共有604名受试者参与。所有患者均报告有鼻出血，285例（47.2%）有毛细血管扩张，545例（90.2%）有HHT家族史；167例（27.6%）患者有轻度鼻出血（ESS<4），285例（47.2%）报告有中度鼻出血（ESS≥4<7），152例（25.2%）报告有重度鼻出血（ESS≥7）。与轻度鼻出血患者相比，重度鼻出血患者的HR-QoL身体成分总结（PCS）和心理成分总结（MCS）得分均较低（p<0.001，p<0.001）。
ESS是HR-QoL的主要决定因素，应被视为HHT相关性鼻出血治疗效果的一项衡量指标。
