Abstract

Abstract Introduction Ehlers-Danlos syndrome (EDS) is an heritable collagen disorder with various multisystemic clinical manifestations affecting primarily skin, ligaments and joints, blood vessels and internal organs. The clinical spectrum is very large from mild skin and joint hypermobility to severe physical disability. Patients with Ehlers-Danlos syndrome often complain of poor sleep quality and fatigue with impaired quality of life. The purpose of this study was to assess any objective sleep disturbances in EDS by polysomnography. Methods In this case-control study, we included 47 patients EDS type III (hypermobile type) (29 F et 18 M) which were one to one strictly matched to 47(29 F et 18 M) controls according to sex, age, and BMI. Participants underwent level-1 polysomnography for a complete sleep study. Results The two group were strictly similar for age and BMI (mean age 29.3 ± 9.2 years, BMI 23.3 ± 4.4 kg/m²). Total Sleep time (TST) was significantly reduced in EDS (343.7 ± 69.3 min versus 395 ± 74.8 min; F= 11.9; p< 0.01). Sleep quality was significantly impaired, with a decreased Sleep efficiency (SE): 74.4 ± 10.5 versus 90.2 ± 7.8 F= 68.5; p< 0.001), an increased wake after sleep onset (WASO) time (116.5 ± 45.7 min versus 43.3 ± 36.8 min; F= 73.2; p <0.01) and micro arousal index (14.8 ±7.8 versus 6.5 ±4.3; F= 41.2; p<0.01). We also found a significant reduction of slow wave sleep length, but REM sleep was not affected. The apnea-hyponea index (IAH) and periodic leg movement index (PLMI) were higher in EDS patients (IAH: 10.8±4.8 versus 5.8±3.9; F= 30.4; p<0.01 et PLMI: 4.5 ±4.6 versus 2.6± 2.5; F= 6; p<0.01). In patients with EDS type III, the prevalence of OSA (AHI>10/hour) was 75% versus 7.1 % in the control group (OR 5.1 (95% CI 2.3 to 14.7); p<0.001). Conclusion PSG may help in better understanding the diagnosis and treatments of EDS patients. Support

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