Semmelweis University
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Budapest, Hungary
Not yet recruiting
Safety and Efficacy of Mirivadelgat Compared with Placebo in Patients with Pulmonary Hypertension Associated with Interstitial Lung Disease
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What is this trial about?
A plain-language summary of the goals, design and what participants do
The study looks at people with Pulmonary Hypertension associated with Interstitial Lung Disease, a condition where scar tissue in the lungs narrows the main blood vessels, raising pressure and making breathing difficult. The experimental medicine being tested is mirivadelgat, an oral capsule that works by activating an enzyme called Aldehyde Dehydrogenase 2, which may help the blood vessels relax and improve blood flow.
The purpose of the study is to see whether the drug can lower the pressure that the heart has to pump against, measured as pulmonary vascular resistance, compared with a placebo. Participants will be randomly assigned to receive either the medicine or the placebo for about three months, with regular visits to check safety, blood tests, and simple physical tests such as the 6-minute walk distance to see how far they can walk in six minutes. The study will also collect information on symptoms, quality of life, and any need for additional treatments.
The research process
The trial runs in 6 steps – from screening to follow-up. Each step says what happens and what the team monitors.
Who can join the trial?
20 criteria
- Clinical diagnosis of PH‑ILD: You must have been told by a doctor that you have pulmonary hypertension (high blood pressure in the lungs) linked to an interstitial lung disease (a group of lung disorders that cause scarring).
- Confirmed interstitial lung disease by a high‑resolution CT scan of the chest (a detailed X‑ray picture) taken within the past 180 days, or by a previous lung tissue sample such as a surgical biopsy or cryobiopsy. Other findings like emphysema are allowed if they are not the main problem on the scan.
- Right‑heart catheterization (RHC) performed during screening showing:
- Pulmonary vascular resistance (PVR) of at least 4 Wood units (a measure of how hard the heart must work to push blood through the lungs).
- Pulmonary capillary wedge pressure (PCWP) of 12 mmHg or less if PVR is 4‑<6.25 Wood units, or 15 mmHg or less if PVR is 6.25 Wood units or higher. If PCWP cannot be measured, the left‑ventricular end‑diastolic pressure (LVEDP) can be used with the same limits.
- Mean pulmonary arterial pressure (PAP) greater than 20 mmHg (average pressure in the lung artery).
- 6‑minute walk test results between 100 meters and 500 meters at the screening visit. This test measures how far you can walk in six minutes and reflects your functional ability.
- Willingness to have a repeat RHC, chest CT scan, and MRI scan before the study ends.
- If you are taking long‑term medicines for your lung disease (such as nintedanib, pirfenidone, or immunosuppressive drugs), the dose must have been stable for at least 30 days before screening and you must have been on the medicine for at least 90 days.
- For participants in Hungary, you must already be receiving a phosphodiesterase‑5 inhibitor (a medication that helps lower lung blood pressure).
- If you use supportive inhalers or other respiratory medicines (for asthma, etc.), the dose must have been stable for at least 30 days before screening.
- You must be able to give your own consent, understand study information, and communicate with the study staff (an interpreter can be used if needed). You also need to attend all study visits and be reachable by a phone.
- Your routine laboratory test results must be within normal limits or no more than 1.5 times the upper limit of normal as defined by the testing lab.
- Pulmonary function test (PFT) requirements at screening:
- Forced vital capacity (FVC) less than 90 % of the predicted value (a measure of the total amount of air you can exhale forcefully).
- Diffusion capacity of carbon monoxide (DLCO) less than 70 % of the predicted value (how well oxygen moves from your lungs into the blood), or less than 70 % after correcting for hemoglobin if needed.
- Negative blood tests for hepatitis B surface antigen and hepatitis C antibody (showing you do not have active infection with these viruses).
- Voluntary signing of an informed consent form.
- Age between 18 and 85 years at the time you sign the consent.
- Agreement to use reliable birth‑control methods during the study unless you are not capable of becoming pregnant. This includes:
- Male participants with female partners who could become pregnant must use contraception during treatment and for at least 96 hours after stopping the study drug.
- Female participants who could become pregnant must use contraception during the study and for 30 days after the last dose.
- Women who are post‑menopausal, have had certain surgeries (such as hysterectomy or removal of both ovaries), or have a medical condition that prevents pregnancy are exempt from the contraception requirement.
Who cannot join the trial?
33 criteria
- Having another type of pulmonary hypertension that is not related to the lung disease being studied, such as blood clots in the lungs, untreated sleep apnea, HIV infection, use of methamphetamine or similar drugs, or other classifications of pulmonary hypertension.
- Having a history of congenital heart disease, even if it was treated or repaired in the past.
- Using tobacco, e‑cigarettes, nicotine products, marijuana, or having a significant problem with drugs or alcohol within the past 6 months.
- Having an acute pulmonary embolism (a blood clot in the lung) within the last 90 days.
- Participating in a pulmonary rehabilitation program within the last 90 days (unless the program was stable for more than 90 days before screening).
- Taking any investigational drug, device, or therapy, or being in another clinical study with treatment intent, within 30 days or five drug half‑lives (whichever is longer) before the first dose of the study medication.
- Having a body mass index (BMI) of 40 kg/m² or higher.
- Having uncontrolled high blood pressure (systolic >160 mmHg or diastolic >100 mmHg) during screening.
- Having another disease that is expected to shorten life to less than 6 months at the time of screening.
- Being judged by the investigator as unable to fully take part in the study or follow the procedures, including problems with addiction or serious mental health conditions.
- Having a high likelihood of needing a lung transplant within 4 months after randomization, as decided by the investigator.
- Showing evidence of significant left‑sided heart disease within the last 6 months, such as:
- Left ventricular ejection fraction (a measure of heart pumping) less than 40%.
- More than mild problems with the heart valves (e.g., moderate or severe mitral or aortic valve disease).
- High pressures in the left side of the heart (LVEDP or PCWP >15 mmHg, or >12 mmHg if pulmonary vascular resistance is high).
- Having a history of serious liver problems, including moderate or severe liver disease (Child‑Pugh B or C), liver enzymes more than three times the normal limit, bilirubin more than twice the normal limit, cirrhosis, or bleeding disorders (INR >1.5, platelet count <70,000).
- Being a female who is pregnant or breastfeeding.
- Having untreated sleep apnea that is worse than mild (5‑14.9 breathing pauses per hour). Treated sleep apnea is allowed.
- Having a prior diagnosis of a connective tissue disease such as systemic sclerosis (scleroderma), lupus, Sjögren’s syndrome, polymyositis/dermatomyositis, antisynthetase syndrome, or rheumatoid arthritis.
- Having an allergic or severe (anaphylactic) reaction to the study drug mirivadelgat or any of its ingredients.
- Having taken mirivadelgat before.
- Having laboratory results outside the allowed range, including:
- Hemoglobin (a measure of red blood cells) less than 10 g/dL.
- White blood cell count less than 3,000 per microliter.
- Platelet count less than 70,000 per microliter.
- Estimated glomerular filtration rate (eGFR, a kidney function test) less than 30 mL/min/1.73 m² or evidence of acute kidney injury.
- Having three or more of the following left‑ventricular risk factors at screening:
- BMI of 30 kg/m² or higher.
- Uncontrolled diabetes (HbA1c >9.5%, high sugar in urine, or diabetic ketoacidosis).
- Significant coronary (heart artery) disease in the past 6 months, such as a heart attack, procedures to open blocked arteries, a bypass surgery, or more than 50% blockage in a major artery.
- Needing 10 liters per minute or more of supplemental oxygen at rest.
- Having received any approved pulmonary hypertension therapy (such as soluble guanylate cyclase inhibitors, endothelin receptor antagonists, or parenteral/oral prostacyclin) within 60 days or five half‑lives before randomization. (Stable use of certain blood pressure medicines like PDE5 inhibitors or inhaled prostacyclin is allowed.)
- Using strong inhibitors or inducers of the enzyme CYP3A4 (for example, certain antiviral drugs, antifungal medicines, some antibiotics, grapefruit juice, St. John’s wort, and many others).
- Having a recent worsening of lung disease or an active lung or upper respiratory infection within 4 weeks of randomization.
- Having a current active cancer (except for localized skin cancers) or a history of cancer that is likely to cause death or require major treatment within the next year.
- Having chronic kidney disease stage IV or worse (eGFR < 30 mL/min/1.73 m²) or evidence of acute kidney injury.
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Institute For Clinical And Experimental Medicine
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Prague, CzechiaUniversity Of Szeged
Verified siteSzeged, Hungary
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Investigated drugs
Mirivadelgat is an oral capsule being tested as a new treatment for patients who have pulmonary hypertension related to interstitial lung disease. It works by activating an enzyme called aldehyde dehydrogenase‑2 (ALDH2), which may help improve the function of blood vessels in the lungs and reduce the pressure the heart has to pump against. In this study, participants take Mirivadelgat for several doses while researchers watch closely for any side effects and measure how well it lowers the resistance in the lung’s blood vessels.
What is already known about the treatment
Mirivadelgat - Mirivadelgat is taken orally as a 300 mg capsule, meaning the patient swallows the pill with water. It is currently in Phase 2 clinical trials, with early studies showing it is safe and being investigated for lung‑related high blood pressure. The drug is intended to treat pulmonary hypertension that occurs with interstitial lung disease, a condition where high pressure in the lung vessels worsens breathing problems. It works by activating the enzyme aldehyde dehydrogenase 2, which helps break down harmful chemicals in cells and improves blood vessel function, and it belongs to the class of enzyme activators.
Investigated diseases
Pulmonary hypertension associated with interstitial lung disease - Pulmonary hypertension associated with interstitial lung disease is a condition where high blood pressure in the lung arteries occurs together with scarring or thickening of lung tissue. The scarring makes it harder for oxygen to pass into the blood, and the increased pressure strains the right side of the heart. Over time the pressure can rise further as the lung tissue becomes more fibrotic, leading to reduced exercise capacity and worsening shortness of breath. The disease often progresses gradually, with symptoms becoming more noticeable during physical activity. It can lead to an increasing need for supplemental oxygen as the lungs become less efficient.
Trial detailsLast updated 21 Oct 2026
Age18+ yearsPhasePhase IITrial ID2026-526703-30-00Protocol codeFP-045C-23-001Estimated enrolment133 patients
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