1021 Obstructive Sleep Apnea and CPAP Efficacy in Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder Patients: A Case-Control Study

Abstract Introduction Hypermobile Ehlers-Danlos syndrome (hEDS) and Hypermobility Spectrum Disorder (HSD) are genetic tissue disorders. They exist on a spectrum with association to various sleep disorders including insomnia and obstructive sleep apnea (OSA), leading to chronic fatigue and daytime sleepiness. Structural and soft tissue abnormalities of the oropharynx in hEDS/HSD patients may exacerbate OSA; these patients may respond differently to continuous positive airway pressure (CPAP). Methods A retrospective review of patients with OSA between 2020 – 2024 at a single institution was conducted. Adults with hEDS/HSD and OSA (cases) were matched based on sex, age, and body mass index (BMI) to adults with OSA alone (controls). Data from polysomnograms (PSG), initial sleep clinic consults, and clinic visits 3-9 months post CPAP were extracted for statistical analysis. The Epworth Sleepiness Scale (ESS) was used to assess daytime sleepiness. Results 68 patients with hypermobility (47% hEDS, 53% HSD, Beighton score 5.71±2.29, age 34.07±13.28, BMI 32.88±8.47) were included as cases with successful matching to 68 controls. All pairs completed sleep clinic consults and polysomnograms; 53 pairs pursued CPAP; 41 pairs were reevaluated in clinic within 3–9 months. The hypermobile cohort exhibited significantly lower sleep efficiency (77%±16% vs 82%±10%, p=0.0396) and a higher prevalence of insomnia (44% vs 34%, p=0.0348). Pre-treatment ESS were similar between cases (10.39±4.89) and controls (11.22±5.12) (p=0.4990). CPAP significantly reduced ESS in controls by 4.46 (p < 0.0001) but had no significant effect in cases, with a decrease of 1.56 (p=0.0620). After treatment, ESS remained higher in cases (8.83±4.47) than controls (6.76±4.15) (p=0.0414). Cases and controls had similar CPAP compliance rates of >4 hours per night (75.6%±25.2% vs 74.6%±26.7%, p = 0.8570), Apnea-Hypopnea Index (16.86±28.09 vs 12.48±16.38, p = 0.2444), and lowest SpO2 (86.08%±4.31% vs 85.67%±3.52%, p = 0.5097). Conclusion CPAP does not significantly improve daytime sleepiness in hypermobile patients despite adequately reducing apnea frequency, suggesting that OSA may not be the primary contributor to chronic fatigue and sleepiness in this patient population. hEDS/HSD should be considered when assessing sleep consolidation and residual sleepiness in response to CPAP therapy. Support (if any)