The Impact of living with Ehlers-Danlos Syndrome on health-related quality of life: A systematic review following the 2017 international EDS classification

Rare Pub Date : 2025-01-01 DOI:10.1016/j.rare.2025.100107

Joanna Foster , Elaine Walklet , Danielle Stephens-Lewis

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Abstract

Objective

The Ehlers-Danlos Syndromes (EDS) are a group of multi-systemic, chronic conditions with complex symptomology. This systematic review aimed to synthesise what is known about the impact of EDS on Health-Related Quality of Life (HRQoL), and associated moderating factors, following a change in diagnostic criteria.

Methods

Fifteen databases, grey literature and reference lists were systematically searched. A systematic review was performed following the Preferred Reporting Items for Systematic Reviews and guidelines for narrative synthesis. Findings were grouped according to outcomes, moderating factors, and measurement instrument. A further synthesis aligned outcomes with domains of HRQoL. Risk of bias was addressed using the Effective Public Health Practice Project assessment tool.

Results

Eight quantitative studies met eligibility criteria. Findings indicate substantial impact due to symptoms and functional status. However, how HRQoL is measured potentially introduces bias such that other factors are overlooked.

Conclusion

This review suggests key aspects of how HRQoL is experienced remain underexplored and underreported. Issues of methodological rigour raise further concerns around the usefulness of study findings. Further research is required to clarify how aspects of HRQoL are prioritised and experienced and how they can best be measured, to improve management of this debilitating condition.

PROSPERO registration number CRD42022318979

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ehers - danlos综合征对健康相关生活质量的影响：2017年国际EDS分类的系统回顾

目的ehers - danlos综合征（EDS）是一组多系统、症状复杂的慢性疾病。本系统综述旨在综合诊断标准改变后EDS对健康相关生活质量（HRQoL）的影响以及相关调节因素。方法系统检索15个数据库、灰色文献和参考文献。按照系统评价的首选报告项目和叙述综合指南进行系统评价。根据结果、调节因素和测量工具对结果进行分组。进一步的综合使结果与HRQoL的结构域一致。使用有效公共卫生实践项目评估工具解决了偏倚风险。结果8项定量研究符合入选标准。研究结果表明，由于症状和功能状态造成了重大影响。然而，衡量HRQoL的方式可能会引入偏差，从而忽略了其他因素。结论：本综述表明，关于如何体验HRQoL的关键方面仍未得到充分探索和报道。方法的严谨性问题引起了人们对研究结果有用性的进一步关注。需要进一步的研究来阐明如何优先考虑和体验HRQoL的各个方面，以及如何最好地测量它们，以改善对这种使人衰弱的疾病的管理。普洛斯彼罗注册号CRD42022318979

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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