Edsivo (celiprolol)
An investigational treatment for Vascular Ehlers-Danlos Syndrome.
The same compound appears under different names depending on the context. Here is how to identify Celiprolol wherever you encounter it, plus the key facts at a glance.
- Generic name
- Celiprolol
- Brand name
- Edsivo
- Drug class
- Selective adrenoreceptor modulator (beta-blocker)
- Manufacturer
- Zevra Therapeutics (formerly Acer Therapeutics)
- How it's taken
- Taken as an oral tablet once daily.
An investigational beta-blocker being studied specifically for vascular Ehlers-Danlos Syndrome (vEDS). Celiprolol blocks beta-1 receptors and partly stimulates beta-2 receptors, a profile that may reduce mechanical stress on fragile blood vessels.
It is already used for vEDS in parts of Europe, and the Phase 3 DiSCOVER trial is underway in the U.S. to support FDA approval.
Where Celiprolol fits
The most advanced medication being developed specifically for vascular EDS. It is used off-label in some countries for vEDS based on the BBEST trial. If approved, it would be the first FDA-approved treatment for vEDS.
How Celiprolol works
In vascular EDS, a mutation in the COL3A1 gene causes defective type III collagen, making arteries and organs dangerously fragile. Celiprolol works in two ways: First, it blocks beta-1 receptors to lower heart rate and reduce the pulsatile force that stresses weakened arterial walls.
Second, unlike standard beta-blockers, it stimulates beta-2 receptors, which appears to promote normal collagen synthesis through the TGF-beta pathway. This dual mechanism targets both the mechanical stress on vessels and the underlying collagen defect.
Mechanism: Selective beta-1 adrenergic blocker with beta-2 agonist activity that reduces arterial wall stress and may promote normal collagen synthesis
Side effects and safety
Side effects are generally mild and manageable. The most commonly reported effects include dizziness, fatigue, and low blood pressure. In the landmark BBEST trial, most side effects were temporary and resolved with dose adjustment. Some patients could not tolerate the full 400 mg target dose due to hypotension or fatigue, but the majority (about 65%) successfully reached full dose.
This is not a complete list of side effects. Talk to your doctor or pharmacist about what to expect and when to seek medical attention.
Taking Celiprolol
Taken as an oral tablet once daily. Treatment typically starts at a low dose (100 mg daily) and is gradually increased over several months to the target dose of 400 mg daily. Dose adjustments are made every 3-6 months based on tolerability. The medication should be taken on an empty stomach (at least 30 minutes before food) for best absorption.
Availability and cost
Only available as the brand-name product.
Not yet commercially available in the U.S. Celiprolol is available as a generic medication in several European countries at relatively low cost. U.S. pricing has not been established pending FDA approval.
Clinical trial results
The BBEST trial (published in 2010) randomized 53 people with vEDS in France and Belgium to celiprolol or no treatment, with blinded review of events. Serious arterial events (ruptures, dissections) occurred in 20% on celiprolol versus 50% without it, and the trial stopped early for benefit. The FDA later judged BBEST not adequate on its own for approval. The Phase 3 DiSCOVER trial (NCT05432466), started in 2022, is a double-blind, placebo-controlled study aiming for about 150 patients; it ends after 46 events, and as of mid-2026 66 people had enrolled.
Development history
Celiprolol has long been used in Europe as a blood pressure medication. Its potential for vEDS was explored after observations that beta-blockers might protect fragile arteries, and the BBEST trial in 2010 provided the first randomized evidence. Acer Therapeutics sought FDA approval under the brand name Edsivo but received a Complete Response Letter in 2019.
The FDA granted Breakthrough Therapy designation in April 2022, and Acer launched the DiSCOVER trial that year under an FDA Special Protocol Assessment agreement. Zevra Therapeutics took over the program when it acquired Acer.
Explore Vascular Ehlers-Danlos Syndrome trials
Common questions about Celiprolol
▸What is Celiprolol (Edsivo)?
An investigational beta-blocker being studied specifically for vascular Ehlers-Danlos Syndrome (vEDS). Celiprolol blocks beta-1 receptors and partly stimulates beta-2 receptors, a profile that may reduce mechanical stress on fragile blood vessels.
It is already used for vEDS in parts of Europe, and the Phase 3 DiSCOVER trial is underway in the U.S. to support FDA approval.
▸How does Celiprolol work?
In vascular EDS, a mutation in the COL3A1 gene causes defective type III collagen, making arteries and organs dangerously fragile. Celiprolol works in two ways: First, it blocks beta-1 receptors to lower heart rate and reduce the pulsatile force that stresses weakened arterial walls.
Second, unlike standard beta-blockers, it stimulates beta-2 receptors, which appears to promote normal collagen synthesis through the TGF-beta pathway. This dual mechanism targets both the mechanical stress on vessels and the underlying collagen defect.
▸What are the side effects of Celiprolol?
Side effects are generally mild and manageable. The most commonly reported effects include dizziness, fatigue, and low blood pressure. In the landmark BBEST trial, most side effects were temporary and resolved with dose adjustment. Some patients could not tolerate the full 400 mg target dose due to hypotension or fatigue, but the majority (about 65%) successfully reached full dose.
▸How is Celiprolol taken?
Taken as an oral tablet once daily. Treatment typically starts at a low dose (100 mg daily) and is gradually increased over several months to the target dose of 400 mg daily. Dose adjustments are made every 3-6 months based on tolerability. The medication should be taken on an empty stomach (at least 30 minutes before food) for best absorption.
▸Is Celiprolol FDA approved?
Celiprolol is currently in phase 3 clinical trials for Vascular Ehlers-Danlos Syndrome. It has not yet received FDA approval.
▸How does celiprolol help in vascular Ehlers-Danlos syndrome?
Celiprolol is a beta-blocker that also partly stimulates beta-2 receptors, which relaxes blood vessels. It is thought to protect fragile arteries in vEDS by lowering the force on vessel walls. In the BBEST trial, fewer people on celiprolol had arterial ruptures or dissections.
▸What clinical evidence supports celiprolol for vEDS?
The BBEST trial in France and Belgium randomized 53 people with vEDS to celiprolol or no treatment and found fewer arterial events (ruptures and dissections) on celiprolol: 20% versus 50%. It was not placebo-controlled, and the FDA did not consider it enough for approval, which is why the placebo-controlled DiSCOVER trial is now running.
▸What is the current regulatory status?
Celiprolol is in Phase 3 development for vEDS in the US, being developed by Zevra Therapeutics. It is already available in some European countries for other indications. It is not yet FDA-approved for any indication in the US.
▸How is celiprolol different from other beta-blockers?
Celiprolol blocks beta-1 receptors, like other beta-blockers, but also partly stimulates beta-2 receptors, which relaxes blood vessels. This profile may be better suited to protecting fragile arteries in vEDS than standard beta-blockers.
▸Is celiprolol available in the US currently?
Celiprolol is not currently FDA-approved or commercially available in the US. Zevra Therapeutics is conducting Phase 3 trials aimed at securing FDA approval specifically for vascular Ehlers-Danlos syndrome.
Sources and references
Every factual claim on this page is drawn from the public sources listed below. Click any reference to open the original document.
- PubMed · 2010-10-30. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome. https://pubmed.ncbi.nlm.nih.gov/20825986/
- ClinicalTrials.gov. DiSCOVER: celiprolol in vascular Ehlers-Danlos syndrome. https://clinicaltrials.gov/study/NCT05432466
- Zevra Therapeutics / SEC · 2026-08. Zevra Therapeutics Form 10-Q for the quarter ended June 30, 2026. https://www.sec.gov/Archives/edgar/data/0001434647/000143464726000068/zvra-20260630.htm