Subacromial space outlet in female patients with multidirectional instability based on hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder measured by ultrasound

There is an association between joint hypermobility, hypermobile Ehlers-Danlos syndrome (hEDS) and different forms of orthostatic intolerance. Objective: to explore autonomic profile in a large cohort of adults with hEDS and hypermobility spectrum disorder (hEDS/HSD) with a multimodal approach. In this observational retrospective study, heart rate, blood pressure and baroreflex sensitivity were estimated in 102 hEDS/HSD subjects during deep breathing, Valsalva maneuver, standing up: 30-15 ratio, Head-Up Tilt and sustained handgrip. Abnormal results and head-up tilt test were common and included postural orthostatic tachycardia syndrome (POTS; 48%), orthostatic intolerance (25.5%) and hypotension (3.9%). Baroreflex sensitivity was significantly different in individuals with POTS compared to the others. This study confirms the high rate and heterogeneity of abnormal autonomic regulation in hEDS/HSD, and suggests the baroreflex sensitivity might distinguish comorbid POTS from other profiles in this subgroup of patients. Abnormal autonomic regulation is common in adults with hEDS/HSD and should be regularly assessed for tailoring the management approach.

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Severity classes in adults with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorder

Rheumatology ◽

10.1093/rheumatology/kez134 ◽

2019 ◽

Vol 58 (10) ◽

pp. 1705-1706

Author(s):

Alan J Hakim

Keyword(s):

Spectrum Disorder ◽

Ehlers Danlos Syndrome ◽

Danlos Syndrome

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Pelvic Girdle Pain, Hypermobility Spectrum Disorder and Hypermobility-Type Ehlers-Danlos Syndrome: A Narrative Literature Review

Journal of Clinical Medicine ◽

10.3390/jcm9123992 ◽

2020 ◽

Vol 9 (12) ◽

pp. 3992

Author(s):

Ahmed Ali ◽

Paul Andrzejowski ◽

Nikolaos K. Kanakaris ◽

Peter V. Giannoudis

Keyword(s):

Pelvic Ring ◽

Patient Reported Outcome Measures ◽

Pelvic Girdle ◽

Spectrum Disorder ◽

Pelvic Girdle Pain ◽

Treatment Modalities ◽

Ehlers Danlos Syndrome ◽

Ongoing Research ◽

Patient Reported ◽

Danlos Syndrome

Pelvic girdle pain (PGP) refers specifically to musculoskeletal pain localised to the pelvic ring and can be present at its anterior and/or posterior aspects. Causes such as trauma, infection and pregnancy have been well-established, while patients with hypermobile joints are at greater risk of developing PGP. Research exploring this association is limited and of varying quality. In the present study we report on the incidence, pathophysiology, diagnostic and treatment modalities for PGP in patients suffering from Hypermobility Spectrum Disorder (HSD) and Hypermobility-Type Ehlers-Danlos Syndrome (hEDS). Recommendations are made for clinical practice by elaborating on screening, diagnosis and management of such patients to provide a holistic approach to their care. It appears that this cohort of patients are at greater risk particularly of mental health issues. Moreover over, they may require a multidisciplinary approach for their management. Ongoing research is still required to expand our understanding of the relationship between PGP, HSD and hEDS by appropriately diagnosing patients using the latest updated terminologies and by conducting randomised control trials to compare outcomes of interventions using standardised patient reported outcome measures.

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