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Cited by 47Obstructive Sleep Apnea ResearchSleep and related disordersConnective tissue disorders research

Obstructive sleep apnoea and quality of life in Ehlers-Danlos syndrome: a parallel cohort study

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Gaisl, Thomas, Giunta, Cecilia, Bratton, Daniel J, Sutherland, Kate, Schlatzer, Christian, Sievi, Noriane, Franzen, Daniel, Cistulli, Peter A +2 more·2017·Thorax
DOI 10.1136/thoraxjnl-2016-209560
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Abstract

BACKGROUND: Patients with the connective tissue disorder Ehlers-Danlos syndrome (EDS) often suffer from fatigue, excessive daytime sleepiness and impaired quality of life. Obstructive sleep apnoea (OSA) may be an underlying cause for these symptoms but its prevalence in this population is unclear. METHODS: In this prospective parallel-cohort study, we included 100 adult patients with EDS (46% hypermobile-type, 35% classical-type and 19% other), which were one-to-one matched to 100 healthy adult controls according to sex, age, weight and height. Participants underwent structured interviews (including short-form 36) and level-3 respiratory polygraphy. OSA was defined as apnoea-hypopnea index ≥5/hour. Photographic craniofacial phenotyping was conducted in a subgroup. Conditional logistic regression was used to compare the prevalence of OSA. RESULTS: In patients with EDS, OSA prevalence was 32% versus 6% in the matched control group (OR 5.3 (95% CI 2.5 to 11.2); p<0.001). The EDS group reported impaired quality of life in all dimensions (p<0.05) and significantly higher excessive daytime sleepiness measured by the Epworth Sleepiness Scale (median (quartiles) 11 (7-14) vs 7 (5-10); p<0.001). OSA severity was positively associated with daytime sleepiness and lower quality of life in the EDS group. There was no evidence of a difference between the two study groups in terms of craniofacial phenotypes. CONCLUSIONS: The prevalence of OSA is higher in patients with EDS than in a matched control group. This is of clinical relevance as it is associated with fatigue, excessive daytime sleepiness and impaired quality of life. Further studies are needed to assess the clinical benefit of OSA treatment in patients with EDS. TRIAL REGISTRATION NUMBER: NCT02435745.

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Authors
Gaisl, T., Giunta, C., Bratton, D. J., Sutherland, K., Schlatzer, C., Sievi, N., Franzen, D., Cistulli, P. A., Rohrbach, M., & Kohler, M.
Affiliations
  • University Hospital Zurich (CH)
  • University Children's Hospital Zurich (CH)
  • The University of Sydney (AU)
  • University of Zurich (CH)
Corresponding author
Kohler, Malcolm
ORCID
Venue
Thorax
Publisher
BMJ
Published
2017-01-10
Volume
72
Issue
8
Pages
729-735
ISSN
  • 0040-6376 (ISSN-L)
  • 1468-3296
DOI
10.1136/thoraxjnl-2016-209560
OpenAlex
W2568542805
Cited by
47
References
7
Citations by year
2026: 3 · 2025: 6 · 2024: 1 · 2023: 3 · 2022: 4 · 2021: 8 · 2020: 8 · 2019: 8 · 2018: 6
FWCI
4.47
Type
journal article
Language
en
Open access
Locations
  • Thorax (publishedVersion)
  • PubMed (publishedVersion)
  • HighWire Press Open Archive (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich) (submittedVersion)
Funders
  • ResMed
  • Medizinische Universität Innsbruck
  • Universität Innsbruck
  • Lungenliga Schweiz
  • Lunge Zürich
Topics
  • Health Sciences › Medicine › Physiology › Obstructive Sleep Apnea Research
  • Social Sciences › Psychology › Experimental and Cognitive Psychology › Sleep and related disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Keywords
obstructive sleep apnea, Ehlers-Danlos syndrome, quality of life, excessive daytime sleepiness, fatigue, connective tissue diseases, hypermobile Ehlers-Danlos syndrome, classical Ehlers-Danlos syndrome, craniofacial phenotype, OSA prevalence, apnea-hypopnea index
MeSH terms
Adult, Ehlers-Danlos Syndrome, Female, Follow-Up Studies, Humans, Male, Middle Aged, Prospective Studies, Quality of Life, Risk Factors, Switzerland, Prevalence, Polysomnography, Sleep Apnea, Obstructive

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