Too high and too loose: dysautonomia and the hypertensive paradox in hypermobility disorders.

Abstract

Purpose of review: Hypertension in hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) represents an increasingly recognized but poorly understood clinical phenomenon. This review addresses the unique pathophysiology, diagnostic considerations, and management challenges of hypertension occurring in the context of dysautonomia - a comorbidity affecting up to 70% of patients with hEDS/HSD.

Recent findings: Emerging evidence identifies distinct hypertensive endotypes in hEDS/HSD, including hyperadrenergic dysautonomia, paroxysmal hypertension, cervicogenic hypertension, and nocturnal/supine hypertension. Pathophysiologic mechanisms involve connective tissue laxity, altered baroreflex function, small fiber neuropathy, and mast cell activation. Standard antihypertensive therapies may paradoxically worsen symptoms by exacerbating orthostatic intolerance, while targeted approaches using alpha-blockers and central sympatholytics show promise in hyperadrenergic phenotypes.

Summary: Management of hypertension in hEDS/HSD requires adaptation of standard guidelines to accommodate dysautonomia-specific considerations, including withdrawal of provocative medications, avoidance of volume-depleting agents, and phenotype-directed pharmacotherapy. Long-term cardiovascular outcomes remain poorly defined, highlighting critical research gaps and the need for evidence-based consensus guidelines tailored to this unique patient population.