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Atrasentan (Vanrafia)

Vanrafia, generic name atrasentan, is a prescription medication that is FDA-approved to treat IgA Nephropathy. This medication is taken alongside your current medications to help slow the progression of your rare kidney disease. It does not suppress the immune system and is not a steroid.

Medically reviewed by Dr. Anjay Rastogi, MD PhD and Dr. Gaia Coppock, MD

How does Vanrafia Work?

Vanrafia works by blocking receptors in the kidney called endothelin-A receptors. These receptors normally help control blood flow in the kidney. In people with IgA nephropathy, the endothelin receptors can become overactive and cause kidney damage. Doctors often monitor this damage by measuring how much protein is spilling into urine (proteinuria). In studies, researchers found that Vanrafia lowered proteinuria by about 40% for people with IgA nephropathy.

How is Vanrafia Administered?

Vanrafia is a pill that is taken once a day with or without food. It should be swallowed and not crushed.

Is Vanrafia Right for Me?

You and your doctor can decide together whether this medication is right for you. In people with IgA nephropathy who have more than 500 mg of protein in their urine, this medication can help to further reduce or lower proteinuria.  Lower proteinuria is associated with preserving kidney function over time.

*Note: The decision to prescribe a medication is the responsibility of your physician/primary care provider based on his/her evaluation of your condition. The above is meant for informational purposes only. Discuss this information and all information about drugs/medications with your physician before starting or stopping any medication.”

Frequently Asked Questions

The ALIGN study was a phase 3, multinational, double-blind, randomized, controlled trial. It evaluated the efficacy of atrasentan (Vanrafia) in reducing proteinuria in patients with IgA nephropathy.  The study included 340 patients from different centers in many different nations.  It was published in The New England Journal of Medicine in 2024. The results of the ALIGN study showed a 36.1% reduction in proteinuria in people taking atrasentan compared to those who were treated with standard of care (blood pressure management alone). The study investigators are still collecting data to understand whether Vanrafia preserves kidney function long term.  Other studies have shown that lowering urine protein below 500 mg in people with IgA nephropathy is associated with improved kidney outcomes over time, and Vanrafia can help to reduce proteinuria.  

Vanrafia is generally well tolerated. The ALIGN study compared side effects from people taking Vanrafia to people who were taking blood pressure management alone (the placebo group).  The most common side effects include: swelling of the hands, legs, ankles, and feet (10% of people taking Vanrafia vs 7% of people in the placebo group); low red blood cells (6% of people taking Vanrafia vs 1% in the placebo group); and increased liver enzymes (2% of people taking Vanrafia vs 1% in the placebo group).

Women who would like to become pregnant should not take Vanrafia as it can cause serious birth defects if taken during pregnancy. Vanrafia may cause decreased sperm counts in some men during treatment. This has not been studied specifically in people with IgA nephropathy but in studies for diabetic kidney disease, men had a lower sperm count while taking Vanrafia. Their sperm counts returned to normal within about 3 months after stopping treatment. 

Both Vanrafia and Filspari block endothelin-A receptors, but Filspari also blocks another receptor called angiotensin II.  Filspari is called a “dual endothelin angiotensin II receptor antagonist” because it blocks two receptors.  Since Vanrafia only blocks one receptor, doctors may recommend also taking a separate pill that blocks angiotensin receptors.  Taking Vanrafia and a separate pill (instead of one pill that blocks both receptors) allows for more flexibility with doses. This can be helpful for people who cannot tolerate the medication.  It may also be helpful for people who only need a lower dose of the medicine that blocks the angiotensin receptor. 

There is also a difference in monitoring between Filspari and Vanrafia.  People who take Filspari need to get monthly labs for the first year of treatment. The FDA does not have a mandate or requirement for how often people taking Vanrafia must have labs.  You and your doctor will decide how frequently to monitor labs while taking Vanrafia.

Vanrafia is FDA-approved for the treatment of IgA nephropathy and therefore should be covered by most insurance plans. There are other financial support options that also may be available through the drug company that can help with medication costs. 

Find a Specialist

It is important to work with a nephrologist who specializes in rare kidney disease (RKD) and can provide personalized advice based on your individual health needs and changes in available treatments. NephCure has a curated list of NephCure Specialists nationwide ready to help you.

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