Vascular Risk Reduction in Patients with Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome arises from defects in type III collagen, causing skin hyperelasticity and fragility and joint hypermobility. Fatal complications, which occur at a median age of 40–50, can include mitral valve prolapse and spontaneous rupture of large- and medium-sized arteries. Although propranolol is known to lower rates of aortic dilatation and death in Marfan syndrome, the preventive role of beta-blockers in Ehlers-Danlos syndrome is unknown. Celiprolol, a β₁-adrenoceptor antagonist with partial β₂-adrenoceptor agonist action, has been used for hypertension in various countries since 1989 but is currently unavailable in the U.S. In an open-label trial with blinded assessment of clinical events, investigators in France and Belgium…