Respiratory Manifestations and Their Physical, Psychological, and Social Impacts in Ehlers-Danlos Syndromes and Generalized Hypermobility Spectrum Disorders: A Narrative Review

Abstract

Ehlers-Danlos Syndromes (EDS) and Generalized Hypermobility Spectrum disorders (G-HSD) are a group of genetic, connective multi-systemic disorders that can affect the musculoskeletal, gastrointestinal, cardiovascular, and respiratory systems. Respiratory manifestations in EDS/G-HSD can contribute to decrements in health-related quality of life (HRQL); however, these relationships have not been previously characterized. We aimed to review the association of respiratory manifestations with the physical, psychological, and social domains of HRQL in EDS/G-HSD. A comprehensive search was conducted using Ovid Medline, Embase, and CINAHL with the following terms: “Ehlers-Danlos Syndrome”, “Hypermobility Spectrum Disorders”, and “Quality of Life”. Selected studies in English that investigated the relationship between respiratory manifestations and HRQL domains in EDS/G-HSD were included in this narrative review from inception to March 2024. Twelve studies described the physical, psychological, or social domains of HRQL relating to respiratory manifestations. Dyspnea, wheezing, and expiratory flow limitations were associated with limitations in physical function and exercise intolerance. Respiratory manifestations were associated with increased fatigue, pain, anxiety, kinesiophobia, and deconditioning. This review highlights the consequences that respiratory manifestations have on the physical domains of HRQL, through limitations on physical activity and exercise. Future studies should aim to identify the impact that respiratory manifestations have on the psychosocial domains of HRQL and develop disease-specific patient-reported measures to evaluate these relationships.