We sought to assess autonomic symptom burden and sexual function in women with HSD/h-EDS and compare their autonomic symptom and sexual dysfunction severity to women with POTS.

Hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos syndrome (h-EDS) are connective tissue disorders associated with joint hypermobility, pain, fatigue and autonomic dysfunction.

This was an IRB-approved cross-sectional community-based study where participants completed the following online questionnaires: (1) COMPASS-31; (2) Female sexual function index (FSFI); (3) Beck Depression Inventory and (4) one additional short form with questions pertaining to comorbidities and sexual activity.

84 female HSD/EDS patients, mean age of 37.1±8.4, participated. 58% reported a concurrent diagnosis of POTS and 25% reported having a triad of POTS, HSD/h-EDS and MCAS. Mean COMPASS-31 score was 51.5±13.8; mean depression score was 24.6 ±11.4, and mean FSFI score was 15.3±7.9. Only 19 (22.6%) women reported having sexual intercourse with a partner in the past 4 weeks, and additional 2 (2.4%) women reported having sexual intercourse with a partner in the past 3 months. Lowest FSFI scores were in the domains of pain (2.1±1.9) and desire (2.2±0.9) followed by arousal (2.6±1.8), lubrication (2.6±2.1), satisfaction (2.8±1.4), and orgasm (2.8±1.9). Compared to patients with POTS, patients with HSD/h-EDS reported greater sexual dysfunction overall and across all domains measured in the FSFI (pain with intercourse, satisfaction, orgasm, arousal, desire, and lubrication).

Women with HSD/h-EDS experience significant autonomic symptom burden and sexual dysfunction, which is greater than in women with POTS. Given its implications in reproduction, relationships, family planning and overall health and well- being, sexual dysfunction represents a significant unmet need that calls for development of targeted diagnostic and therapeutic approaches in the care of patients with HSD/h- EDS.