Prevalence of Obstructive Sleep Apnea in Joint Hypermobility Syndrome: A Systematic Review and Meta-Analysis

Abstract

Study Objectives: Because of associated abnormalities affecting connective tissue in various organs including airways, hypermobility syndrome has been associated with high risk for the development sleep apnea. Ehlers-Danlos syndrome (EDS) and Marfan syndrome (MFS) represent the most common hypermobility syndromes; therefore, the purpose of this review was to examine the prevalence of obstructive sleep apnea (OSA) in these populations. Methods: All publications and poster presentations written in English found through August 2018 that describe the prevalence of sleep apnea among people with EDS or MFS were included. Results: A total of 13 studies were identified, 7 for EDS and 6 for MFS. A combined random prevalence rate of OSA across both populations was 48.9% (95% confidence interval 38.3-59.6), with a slightly higher rate of 59.7% (39.7-77.0) for MFS versus 39.4% (28.8-51.1) for EDS. However, a high degree of heterogeneity across studies was found in both groups (EDS group: Q = 28.6 and I 2 = 79.0; MFS group: Q = 37.1 and I 2 = 86.5). When directly compared to the general population, patients with EDS/MFS were on average six times more likely (odds ratio 6.28 [95% confidence interval 3.31-11.93], P < 0.001, Z = 5.61) to have a diagnosis of OSA. Conclusions: OSA is a previously underestimated EDS/MFS-related complication. The high prevalence of OSA might be the result of bony and soft-tissue abnormalities associated with these hypermobility syndromes. Untreated OSA is thought to worsen cardiovascular complications especially among those with MFS. Further research is needed to better delineate whether the prevalence of OSA is moderated by factors such as sex, body mass index, bony structure, and disorder subtype.

Physical activity and sleep in patients with hypermobile Ehlers–Danlos syndrome and patients with generalized hypermobility spectrum disorder

“…By contrast, previous research in subjects with EDS showed a higher prevalence of obstructive sleep apnea measured by polysomnography or respiratory polygraphy [14,16,18]. However, to our knowledge, no other studies concerning sleep measured by accelerometry in adults with hEDS and G-HSD have been performed with which we can compare our results.…”

“…In turn, reduced physical activity may lead to impaired sleep quality in hEDS/HSD, as these are bidirectionally related in the general population [11][12][13]. Moreover, impaired sleep quality has been observed in patients with symptomatic GJH, reflected as a higher prevalence of obstructive sleep apnea and snoring, problems of maintaining sleep and not feeling refreshed in the morning [14,15]. Gaisl et al (2017) have shown that these sleeping problems have a major impact on their health-related quality of life [16].…”

Pathophysiology of the comorbidity of glaucoma with obstructive sleep apnea: A postulation

“…Similarly, in OSA, the suppleness of tissues in upper airways makes them more collapsible that might also be the reason for its increased association with FES. Furthermore, OSA is common in connective tissue disorders, 54,55 as indicated from its association with Ehler Danlos and Marfan's syndrome, 56 Loeys-Dietz syndrome, 57 and Cutis-laxa associated with hereditary amyloidosis. 58 Thus, connective tissue disorders may predispose patients to OSA, which is associated with a structural alteration of the extracellular matrix of upper airway tissue, and a positive correlation has been reported between elastin fiber network disorganization score and apnea-hypopnea index.…”

Obstructive Sleep Apnea, Palatal Morphology, and Aortic Dilatation in Marfan Syndrome Growing Subjects: A Retrospective Study

“…Within the noncardiovascular features, obstructive sleep apnea (OSA) seems to be highly prevalent in Marfan syndrome. The prevalence rate of OSA reported is 59.7% among adults (Apnea Hypopnea Index cutoff of five events/h) [ 7 ] and 80% among children (Apnea Hypopnea Index cutoff of one event/h) [ 8 ]. In Marfan syndrome patients, the underlying cause of OSA is not well elucidated but might be related to specific craniofacial features and higher upper airway collapsibility [ 9 ].…”