The Impact of Living with Ehlers-Danlos Syndrome on Health-Related Quality of Life: A Systematic Review.

AbstractObjective: 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 boundaries.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 on HRQoL, particularly in how symptoms are perceived and functional status. However, how HRQoL is measured and researched are influencing these results. 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