Introduction
For those living with Vascular Ehlers-Danlos Syndrome (VEDS) or supporting loved ones with this condition, staying updated on research developments can provide valuable information. New studies continue to emerge that help us better understand how medications might reduce the risk of serious complications in VEDS. Here, we share summaries of two recent research articles that provide important insights into potential treatment approaches for our community. These findings are particularly timely as they have implications for the DiSCOVER trial, currently the only FDA-approved human clinical trial specifically for VEDS in the United States.
Celiprolol’s Effect on Blood Pressure Stability
A small pilot study published in Hypertension Research looked into how celiprolol affects blood pressure (BP) in patients with genetically confirmed vascular Ehlers-Danlos syndrome (VEDS). The study included 20 patients who were monitored using 24-hour ambulatory blood pressure monitoring (ABPM), which measures BP at regular intervals throughout the day and night.
The study found that celiprolol treatment did not significantly change average systolic BP, diastolic BP, or pulse pressure (PP) overall from the start of treatment to the highest dose reached. However, the researchers did observe an effect on short-term pulse pressure variability. Pulse pressure is the difference between the top (systolic) and bottom (diastolic) BP numbers. Variability refers to how much these numbers fluctuate over 24 hours.
The results showed that in patients who initially had higher levels of PP variability, celiprolol treatment seemed to reduce this variability. The decrease in variability was proportional to how high the variability was at the beginning. The study also noted that two of the four patients who experienced vascular events during the follow-up period had particularly high measurements of short-term PP variability.
It’s important to remember that this was a preliminary study with a very small number of participants. The researchers acknowledge several limitations, including its retrospective design and the fact that some patients were taking other blood pressure medications, which could affect the results. Therefore, while the findings raise questions about the potential role of PP variability, the authors state that larger, dedicated studies are needed to confirm these observations and understand if reducing PP variability is a specific way celiprolol might work in VEDS. The study did not find conclusive evidence from this small cohort on celiprolol’s overall impact on average BP numbers.
Combining Medications for Enhanced Protection
A second article, published in 2025 by Jeunemaitre and colleagues in Circulation, presents findings from the ARCADE trial (Angiotensin II Receptor Blockade in Vascular Ehlers-Danlos Syndrome). This was the first multicenter, randomized, double-blind, placebo-controlled trial testing the addition of irbesartan—an angiotensin II receptor blocker—to standard celiprolol therapy in patients with VEDS.
The researchers studied 57 patients with genetically confirmed VEDS over a two-year period. Half received irbesartan along with their usual celiprolol treatment, while the other half received a placebo plus celiprolol. Patients receiving both medications experienced nearly 50% fewer arterial events (both symptomatic and asymptomatic) compared to those on celiprolol alone. Specifically, arterial complications occurred in 27.6% of patients receiving both medications versus 53.6% of those receiving only celiprolol.
The combined treatment appeared to provide protection throughout the arterial system, reducing the progression of existing arterial lesions and delaying the onset of new complications. While irbesartan did cause some episodes of low blood pressure (particularly in women), this side effect was manageable through dose adjustments without having to stop the medication entirely.
This study suggests that combining celiprolol with irbesartan could significantly improve outcomes for people with VEDS compared to celiprolol alone.
Relevance to the DiSCOVER Trial
These research findings are relevant, as the DiSCOVER trial (Decentralized Study of Celiprolol on VEDS-related Event Reduction) is currently enrolling participants across the United States. This Phase 3 clinical trial aims to definitively evaluate celiprolol’s effectiveness in reducing life-threatening complications in people with genetically confirmed VEDS. Please note, all potential enrollees will have their genetic mutation confirmed as part of the enrollment process.
The DiSCOVER trial aims to provide the robust data needed for potential FDA approval of celiprolol specifically for VEDS in the United States. The decentralized study design allows participants to complete many aspects of the trial from home, making participation more accessible for patients regardless of proximity to specialty centers.
If you or a loved one has genetically confirmed VEDS with a COL3A1 mutation and are interested in learning more about potentially participating in this important research, more information is available at discoverceliprolol.com.
Conclusion
As our community continues to advocate for more research and better treatment options, staying informed about these developments helps us all make more educated decisions about our care. The participation of people with VEDS in clinical trials is important for advancing our knowledge and improving care for future generations affected by this condition.
Note: Always consult with your healthcare provider before making any changes to your treatment plan. The information in this blog post is not intended as medical advice.
