Clinicians Recommend Screening for Arterial Diseases in Vascular EDS Patients

Clinicians highlighted the importance of regular screening for arterial diseases in patients with Ehlers-Danlos syndrome (EDS) in connection with Aortic Dissection Awareness Day last month.

Arterial diseases — which affect blood vessels carrying blood from the heart to other organs — are known to occur in some of the rarest types of EDS. These conditions can affect arteries of different sizes, from the smallest to the largest, which is the aorta that runs from the left ventricle of the heart down to the abdomen.

Aortic disease primarily affects the vascular type of EDS (vEDS), considered the most severe form of the disorder. Common vEDS symptoms include easy bruising; thin, translucent skin; characteristic facial appearance; and fragile arteries, muscles, and internal organs.

Aortic dissection is one of the most common forms of arterial diseases in vEDS patients. The condition is characterized by a tear in the inner layer of the aorta, causing this artery to swell and potentially burst if medical attention is not sought quickly.

“Aortic and other arterial dissection is a major complication and a cause of death in vascular EDS,” Alan Hakim, director of education for the Ehlers-Danlos Society, said in a press release.

He also emphasized the importance of being aware of the disease and its symptoms, saying, “By raising awareness, we hope to improve patient outcomes and save lives.”

The Ehlers-Danlos Syndrome International Consortium Vascular Committee (EDSICVE) has developed a set of recommendations to improve clinical outcomes in vEDS patients in case of an aortic or other arterial dissection.

Specifically, the committee recommends that patients be able to identify the signs and symptoms of an arterial dissection so they know when to seek immediate medical care. Even if no symptoms are observed, the committee advises that vEDS patients be regularly screened for possible arterial diseases. It also suggests keeping blood pressure at low to normal levels because it can reduce strain on the arterial wall.

Patients should have an informed medical care team and an established emergency protocol to be used in case of a vascular event, according to the guidelines.

The Ehlers-Danlos Society is committed to raising awareness, contributing to education, and supporting research for arterial diseases by working closely with the EDSICVE and the vEDS Collaborative, an organization dedicated to uniting patients, caregivers, family members, and care providers with the goal of advancing vEDS research by building collaborations.

“We are honored to work with world-class researchers, clinicians, and patient organizations in the vEDS Collaborative as we seek to increase early detection of aortic and arterial dissection and improve patient outcomes. Together, we can push forward to save and improve lives,” said Lara Bloom, international executive director of the Ehlers-Danlos Society.

Aortic Dissection Awareness Day was recognized at several events, ranging from medical conferences to patient gatherings, around the world, from Fort Worth, Texas, to Stockholm, Sweden, and Dhaka, Bangladesh. A full list of events can be found here.