Drug therapy for vascular EDS patients

Drug Therapy

The major target of medical intervention has been the maintenance of blood pressure in the normal or low normal range and prevention of surges in blood pressure with the intent to minimise the likelihood of arterial dissection or rupture.

Blood pressure monitoring on a regular basis is recommended to allow for early treatment if hypertension develops, thus reducing the risk for vascular stress and injury.

Doctors may suggest use of blood pressure medications to patients with vascular EDS (vEDS) even if the blood pressure is not high to try to minimise baseline vascular stress and influence the integrity of the blood vessel wall. Discussion regarding medications usually starts with vEDS patients in their early teenage years.

There is currently limited evidence for the use of drug therapy to reduce vascular events. The EDS Service in Sheffield reported on the incidence of vascular events in 126 patients (statistical analysis cohort) in their care and the use of medication. Their retrospective data showed that those patients on a long-term angiotensin II receptor blocker (ARB) and/or beta-blocker (BB) had fewer vascular events than those not on cardiac medication who received the same lifestyle and emergency care advice.

Patients with vEDS may be offered a combined therapy with an ARB and Beta Blocker. Patients are made aware of the limitations of this approach, until such time as more trial data is available.

These are prescription-only medicines, which means they can only be prescribed by a GP or another suitably qualified healthcare professional.

If you are considering drug therapy please talk to your cardiologist and/or genetic counsellor.

Beta-Blockers (BB)

Beta blockers work mainly by slowing down the heart. They do this by blocking the action of hormones like adrenaline. Beta blockers usually come as tablets.

Commonly used beta blockers in patients with vEDS include bisoprolol, celiprolol, atenolol, metoprolol, propranolol and others.

Angiotension Receptor Blockers (ARBs)

Angiotensin receptor blockers (ARBs), also known as angiotensin II receptor antagonists, are used to treat high blood pressure.

These medications relax the blood vessels to allow the blood to flow easily, reducing the workload of your heart and make it easier for your heart to pump blood around your body.

Commonly used ARB's in patients with vEDS include losartan, irbesartan and others.

Publication

Current Evidence and Future Perspectives in the Medical Management of Vascular Ehlers–Danlos Syndrome: Focus on Vascular Prevention.

Abstract: Vascular Ehlers–Danlos syndrome (vEDS) is a rare autosomal dominant connective tissue disease resulting from pathogenic variants in the collagen type III alpha 1 chain (COL3A1) gene, encoding type III procollagen. Patients with vEDS present with severe tissue fragility that can result in arterial aneurysm, dissection, or rupture, especially of medium-caliber vessels.

Although early reports have indicated a very high mortality rate in affected patients, with an estimated median survival of around 50 years, recent times have seen a remarkable improvement in outcomes in this population.

This shift could be related to greater awareness of the disease among patients and physicians, with improved management both in terms of follow-up and treatment of complications. Increasing use of drugs acting on the cardiovascular system may also have contributed to this improvement. In particular, celiprolol, a β1 cardio-selective blocker with a β2-agonist vasodilator effect, has been shown to reduce rates of vascular events in patients with vEDS. However, the evidence on the true benefits and possible mechanisms responsible for the protective effect of celiprolol in this specific setting remains limited.

Drugs targeting the extracellular matrix organization and autophagy–lysosome pathways are currently under investigation and could play a role in the future.

This narrative review aims to summarize current evidence and future perspectives on vEDS medical treatment, with a specific focus on vascular prevention.

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