Department of Prosthodontics, University Hospital Münster, Albert-Schweitzer-Campus 1, Building W30, Münster, D-48149, Germany.
Department of Oral and Maxillofacial Surgery, University Hospital Münster, Münster, 48149, Germany.
Orphanet J Rare Dis. 2023 Sep 19;18(1):294. doi: 10.1186/s13023-023-02877-1.
The Ehlers-Danlos syndromes are a group of clinically and genetically heterogeneous hereditary diseases affecting the connective tissue. They are characterized by hypermobility of the joints, hyperextensible skin and friable tissue. According to current classification, 13 subtypes can be distinguished, of which the hypermobile and the classical subtype are the most prevalent. This study aimed to evaluate patients with classical (cEDS) and hypermobile (hEDS) Ehlers-Danlos syndrome regarding temporomandibular disorder (TMD), chronic pain, and psychological distress.
Support groups from Germany, Austria, and Switzerland were contacted, and social media were used to recruit participants. Free text questions, the German version of the Depression Anxiety and Stress Scale (DASS), and the German version of the Graded Chronic Pain Status (GCPS) were used.
259 participants were included (230 hEDS/29 cEDS). At least 49.2% of the participants had painful or restricted jaw movements, and at least 84.9% had pain in the masticatory muscles, with 46.3% already having a diagnosed TMD. Multivariate analysis showed a significant correlation between TMJ involvement and chronic pain with a 2.5-fold higher risk of chronic pain with a diagnosed TMD. 22.8% of participants had a critical score for depression, 53.3% had a critical score for anxiety, and 34.0% had a critical score for stress.
There is a high prevalence of TMD problems and chronic pain in patients with cEDS and hEDS. The lack of knowledge about these problems can create psychological distress. More research is needed to provide adequate treatment for patients with EDS.
埃勒斯-当洛斯综合征是一组影响结缔组织的具有临床和遗传异质性的遗传性疾病。其特征为关节过度活动、皮肤伸展过度和组织脆弱。根据目前的分类，可区分 13 个亚型，其中过度活动型和经典型最为常见。本研究旨在评估经典型（cEDS）和高活动型（hEDS）埃勒斯-当洛斯综合征患者的颞下颌关节紊乱（TMD）、慢性疼痛和心理困扰。
联系了德国、奥地利和瑞士的支持小组，并利用社交媒体招募参与者。使用自由文本问题、德国版抑郁焦虑和压力量表（DASS）以及德国版慢性疼痛分级状况（GCPS）。
共纳入 259 名参与者（230 名 hEDS/29 名 cEDS）。至少 49.2%的参与者有颌骨运动疼痛或受限，至少 84.9%的参与者咀嚼肌疼痛，46.3%的参与者已被诊断为 TMD。多变量分析显示 TMJ 受累与慢性疼痛显著相关，TMD 诊断的慢性疼痛风险增加 2.5 倍。22.8%的参与者抑郁评分临界，53.3%的参与者焦虑评分临界，34.0%的参与者压力评分临界。
cEDS 和 hEDS 患者 TMD 问题和慢性疼痛的患病率较高。对这些问题的认识不足会导致心理困扰。需要进一步研究为 EDS 患者提供适当的治疗。
