Department of Psychology, Anglia Ruskin University, Cambridge, UK.
Faculty of Medecine, University Paris-Est Créteil, Créteil, France.
Disabil Rehabil. 2020 Dec;42(25):3679-3686. doi: 10.1080/09638288.2019.1608595. Epub 2019 May 7.
Among the Ehlers-Danlos syndromes, the hypermobile subtype (hEDS) is the most common. The variety, accumulation and duration of the painful symptoms make hEDS a chronic and highly disabling condition. Identifying drivers of pain and mobility disability in hEDS is necessary to provide adapted prevention and intervention programs. A cross-sectional sample of 75 adults with hEDS according to Villefranche criteria (67 women; 36 years, SD = 11.2) responded to self-assessment questionnaires regarding psychological (negative thoughts and emotions), health (pain, fatigue, diagnostic delay, body mass index, and mobility disability) and socio-demographic variables. Forty-three percent of the participants suffer from severe pain, and two thirds suffer from a high level of mobility disability. Multivariate analyses show that delay of diagnosis, being professionally active and thoughts of helplessness are variables that increase the likelihood of severe pain, whereas age, fatigue, and body mass index predict mobility disability. These results confirm previous research concerning the high disease burden observed in hEDS patients, and highlight the importance of multidisciplinary management that includes nutritional and psychological advice to address this pathology. Increasing awareness of the clinical aspects of hEDS among physicians is necessary to reduce diagnostic delay and minimize negatives outcomes.Implications for RehabilitationA high proportion of patients with hypermobile Ehlers-Danlos syndrome suffer from severe pain (>42%) and a high level of mobility disability (>65%).Different psychosocial and health variables predict pain and mobility disability in patients with hypermobile Ehlers-Danlos syndrome.Awareness concerning the clinical picture of hypermobile Ehlers-Danlos syndrome among health care professionals is necessary to reduce diagnosis delay and the burden of the disease.
在埃勒斯-当洛斯综合征中，高活动型亚型（hEDS）最为常见。疼痛症状的多样性、积累和持续时间使得 hEDS 成为一种慢性且高度致残的疾病。确定 hEDS 中疼痛和活动障碍的驱动因素对于提供适应性的预防和干预方案是必要的。根据 Villefranche 标准，75 名 hEDS 成年人（67 名女性；36 岁，标准差=11.2）的横断面样本对自我评估问卷做出了回应，这些问卷涉及心理（负面想法和情绪）、健康（疼痛、疲劳、诊断延迟、体重指数和活动障碍）和社会人口统计学变量。43%的参与者患有严重疼痛，三分之二的参与者活动障碍程度较高。多变量分析表明，诊断延迟、从事职业活动和无助感是增加严重疼痛可能性的变量，而年龄、疲劳和体重指数则预测活动障碍。这些结果证实了先前关于 hEDS 患者观察到的高疾病负担的研究，并强调了包括营养和心理建议在内的多学科管理对解决这种病理的重要性。提高医生对 hEDS 临床方面的认识对于减少诊断延迟和最小化负面结果是必要的。
康复意义
相当一部分患有高活动型埃勒斯-当洛斯综合征的患者患有严重疼痛（>42%）和高度活动障碍（>65%）。
不同的心理社会和健康变量预测高活动型埃勒斯-当洛斯综合征患者的疼痛和活动障碍。
提高医疗保健专业人员对高活动型埃勒斯-当洛斯综合征临床特征的认识，对于减少诊断延迟和疾病负担是必要的。
