Autonomic and sensory dysfunction in hypermobile Ehlers-Danlos syndrome: How do small fibers contribute?

Abstract

Hypermobile EDS (hEDS) is associated with joint hypermobility, early-onset osteoarthritis, chronic pain, and dysautonomia. This study explored sensory-vascular profiles, as both nociceptive and autonomic nerve fibers are part of the small-fiber group. The aim was to examine sensory-vascular characteristics in an hEDS family, with and without dysautonomia. Five female participants (ages 12–60) underwent tilt-table testing, heart-rate-variability analysis, cutaneous vasoconstriction testing, thermal sensory- and microcirculation testing. Four reported pain; two had dysautonomia and vascular hyperelasticity. All exhibited A-delta fiber loss, while those with dysautonomia also showed C-fiber impairment. Findings indicate dysautonomia and vascular maladaptation worsens with small-fiber dysfunction in hEDS.