PDF(Pubmed)
Abstract：
Joint hypermobility (JHM) is a common physical trait. It may occur alone or in combination with musculoskeletal (MSK) pain, outside or within more complex phenotypes. Hypermobility spectrum disorders (HSD) are diagnosed in individuals with JHM and related MSK pain, when an alternative diagnosis cannot be identified. Conversely, the Ehlers-Danlos syndrome (EDS) encompasses a group of rare hereditary connective tissue disorders featuring JHM along with other pleiotropic manifestations. The 2017 EDS Classification identifies 13 different subtypes. Hypermobile EDS (HEDS) is the only EDS variant still lacking a confirmatory test.
Literature was reviewed searching for the most relevant papers related to key arguments. Particular attention was focused on papers published after the 2017 Classification.
Definition, epidemiology, assessment tools and patterns of JHM are presented. The morbid nature of the 2017 EDS Classification and of the \'spectrum\' is also illustrated.
We discuss current limitations and disagreements concerning the \'spectrum\', HSD and HEDS.
In the clinical context, elucidation of the pathophysiology of pain related to JHM should develop in parallel with the analysis of pleiotropic manifestations of syndromes with JHM.
Future challenges concerning classification, nosology, diagnosis and management of JHM, EDS and related disorders are discussed.
Literature was reviewed searching for the most relevant papers related to key arguments. Particular attention was focused on papers published after the 2017 Classification.
Definition, epidemiology, assessment tools and patterns of JHM are presented. The morbid nature of the 2017 EDS Classification and of the \'spectrum\' is also illustrated.
We discuss current limitations and disagreements concerning the \'spectrum\', HSD and HEDS.
In the clinical context, elucidation of the pathophysiology of pain related to JHM should develop in parallel with the analysis of pleiotropic manifestations of syndromes with JHM.
Future challenges concerning classification, nosology, diagnosis and management of JHM, EDS and related disorders are discussed.
摘要：
背景：关节过度活动（JHM）是一种常见的身体特征。它可以单独发生或与肌肉骨骼（MSK）疼痛相结合，在更复杂的表型之外或之内。在患有JHM和相关MSK疼痛的个体中诊断出高迁移率频谱障碍（HSD），当无法确定替代诊断时。相反，Ehlers-Danlos综合征(EDS)包括一组以JHM和其他多效性表现为特征的罕见遗传性结缔组织疾病。2017年EDS分类识别了13种不同的亚型。HypermobileEDS（HEDS）是唯一仍缺乏确证测试的EDS变体。
方法：文献综述寻找与关键论点相关的最相关的论文。特别关注2017年分类后发表的论文。
结果：定义，流行病学,介绍了JHM的评估工具和模式。还说明了2017年EDS分类和“光谱”的病态性质。
结果：我们讨论当前关于“频谱”的限制和分歧，HSD和HEDS。
结论：在临床背景下，与JHM相关的疼痛病理生理学的阐明应与JHM综合征的多效性表现的分析同时进行。
结论：关于分类的未来挑战，nosology,JHM的诊断和管理，讨论了EDS和相关疾病。
方法：文献综述寻找与关键论点相关的最相关的论文。特别关注2017年分类后发表的论文。
结果：定义，流行病学,介绍了JHM的评估工具和模式。还说明了2017年EDS分类和“光谱”的病态性质。
结果：我们讨论当前关于“频谱”的限制和分歧，HSD和HEDS。
结论：在临床背景下，与JHM相关的疼痛病理生理学的阐明应与JHM综合征的多效性表现的分析同时进行。
结论：关于分类的未来挑战，nosology,JHM的诊断和管理，讨论了EDS和相关疾病。
