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A Phase 2 Study Evaluating the Safety and Efficacy of Oral NS-863 in Adults 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

Participants with Pulmonary Hypertension Associated with Interstitial Lung Disease (PH-ILD) have high blood pressure in the lungs caused by scarring and thickening of lung tissue, which makes breathing difficult and can strain the heart. The study examines the oral medication NS-863, given as a film‑coated tablet, and compares it with a matching inactive pill (placebo) to see if it can improve the condition.

The purpose of the study is to evaluate how well NS-863 works and how safe it is compared with placebo in people with this disease. Participants are randomly assigned to receive either the active drug or the placebo, taken once daily for several weeks. During the trial, they attend regular visits where blood samples, heart rhythm checks, and breathing tests are performed, and they complete simple questionnaires about symptoms. Simple physical tests, such as a walking distance test, are used to track changes over time.

Key outcomes include changes in pulmonary vascular resistance, which measures the pressure the heart must overcome to pump blood through the lungs, and functional measures such as the 6-minute walk distance (6MWD), a short walking test that reflects overall stamina. Participants also rate breathlessness using the Borg dyspnea scale, and their disease severity is classified with the World Health Organization Functional Classification (WHO FC). Heart health is monitored with an echocardiogram, and quality of life is assessed using the King's Brief Interstitial Lung Disease (KBILD) questionnaire. Detailed images of the lungs are obtained with high-resolution computed tomography to observe any changes in tissue scarring.

The research process

The trial runs in 7 steps – from screening to follow-up. Each step says what happens and what the team monitors.

  1. Step 1

    Randomization and Receipt of Study Medication

    After joining the study, you will be randomly assigned to receive either NS-863 or a matching placebo. The assignment is unknown to both you and the study team (double‑blind).

    NS-863 is provided as a film‑coated tablet containing 232 mg of the active substance and is taken by mouth. The placebo looks the same but contains no active drug.

  2. Step 2

    Baseline Assessments

    Before you start taking the medication, a series of tests will be performed to record your health status.

    These tests include measurement of vital signs (blood pressure, heart rate), blood sampling for laboratory analysis, a 12‑lead electrocardiogram (ECG) to check heart rhythm, high‑resolution computed tomography (CT) imaging of the lungs, pulmonary function tests, and a test of lung diffusion capacity for carbon monoxide.

    You will also complete a 6‑minute walk test (6MWD) to measure exercise capacity, answer questions about breathlessness using the Borg dyspnea scale, and be evaluated with the World Health Organization Functional Classification (WHO FC) and the King's Brief Interstitial Lung Disease questionnaire (KBILD).

  3. Step 3

    Daily Medication Intake

    You will take the assigned study tablet by mouth each day for the duration of the treatment period, following the schedule given by the study staff.

    The medication is taken at the same time each day to maintain consistent drug levels.

  4. Step 4

    Scheduled Clinic Visits for Safety and Efficacy Checks

    During the trial you will attend regular clinic visits. At each visit the study team will repeat many of the baseline assessments to monitor your condition and any effects of the medication.

    Assessments include vital signs, blood tests, ECG, lung imaging, pulmonary function tests, the 6‑minute walk test, Borg dyspnea scale, WHO FC, and KBILD questionnaire.

    These visits also allow the team to record any adverse events (unwanted medical occurrences) and to evaluate changes in pulmonary vascular resistance (PVR), which is the primary measure of how well the drug works.

  5. Step 5

    Monitoring for Adverse Events

    Throughout the study you are asked to report any new symptoms, side effects, or health problems to the study staff.

    All reported events are classified and reviewed, including treatment‑emergent adverse events, serious adverse events, and events of special interest.

  6. Step 6

    End‑of‑Treatment Assessment

    When the planned treatment period ends, a final set of assessments identical to the baseline and interim visits will be performed.

    The final assessment captures the overall change in PVR, exercise capacity, lung function, and safety parameters.

  7. Step 7

    Post‑Treatment Follow‑Up

    After stopping the study medication, you will have a follow‑up visit to ensure that any lingering effects are identified and to complete any remaining safety evaluations.

    No further study medication is taken after this visit.

Who can join the trial?

9 criteria

  • You must be able to read, understand, and sign a written informed consent form before joining the study.
  • You need to be an adult, aged 18 to 80 years old (19 to 80 years old if you live in South Korea).
  • You must have a confirmed diagnosis of WHO Group 3 pulmonary hypertension linked to interstitial lung disease (ILD) or combined pulmonary fibrosis and emphysema (CPFE). This must be shown by a special chest scan called HRCT imaging that was done within the last 6 months. For CPFE, the emphysema portion must be 15 % or less and the lung scarring (ILD) must be greater than the emphysema.
  • You need to have had a right‑heart catheterization (RHC) within 35 days before the first dose and meet all three measurements:
    • Pulmonary vascular resistance (PVR) greater than 4 Wood units.
    • Pulmonary artery wedge pressure (PAWP) or left ventricle end‑diastolic pressure (LVEDP) of 15 mm Hg or less.
    • Mean pulmonary artery pressure (mPAP) greater than 20 mm Hg.
    • You must be able to walk between 100 and 500 meters in a 6‑minute walk distance (6MWD) test. The test is done twice (at least 4 hours apart) and the two results must be within 15 % of each other.
    • If you have a connective‑tissue disease, your forced vital capacity (FVC) must be less than 70 % of the normal predicted value.
    • You must be able to attend all scheduled study visits and follow the instructions given by the study doctor.

Who cannot join the trial?

19 criteria

  • Having a diagnosis of pulmonary arterial hypertension (PAH) or other types of pulmonary hypertension (PH) that are not caused by the specific lung disease being studied (known as WHO Group 3 PH‑ILD); PAH means high blood pressure in the arteries of the lungs, and PH means high blood pressure in the lungs overall.
  • Having any other serious illness that the doctor thinks could put you at risk, affect the study results, or stop you from staying in the trial for the whole time.
  • Having significant left‑sided heart disease, defined as a pulmonary artery wedge pressure (PAWP) greater than 15 mm Hg (a pressure measurement) or a left‑ventricular ejection fraction (LVEF) less than 40 % (a measure of how well the left side of the heart pumps).
  • Needing more than 6 liters per minute of supplemental oxygen at rest when the study starts.
  • Having a serious problem with drug or alcohol use in the past 6 months, as judged by the doctor.
  • Having mental health problems or other conditions that make it hard to follow the study instructions.
  • Having a history of urinary stones (kidney or bladder stones).
  • Having had an acute kidney injury (a sudden loss of kidney function) within the past 12 weeks.
  • Having blood in the urine (hematuria) with more than 5 red blood cells per high‑power field, or having protein in the urine above certain levels (urine albumin‑creatinine ratio (UACR) > 150 mg/g or urine protein‑creatinine ratio (UPCR) > 400 mg/g) at screening.
  • Starting a pulmonary or cardiac rehabilitation program within the past 12 weeks.
  • Having a life expectancy (expected remaining lifespan) of less than 6 months.
  • Having diseases or symptoms that would make the six‑minute walk test (6MWT) difficult, such as poor blood flow to the limbs, joint or muscle problems, or severe obesity.
  • Currently smoking cigarettes or e‑cigarettes, or having quit within the past 6 months, if you have smoked at least 20 pack‑years (equivalent to one pack per day for 20 years).
  • Being pregnant, breastfeeding, or planning to become pregnant during the study.
  • Using two platelet‑inhibiting medicines at the same time (dual platelet inhibitor therapy).
  • Having a severe acute or chronic medical problem or lab abnormality that raises risk, such as a history of bleeding in the brain (intracranial hemorrhage) or other bleeding events within the past 6 months, or having a very low white‑blood‑cell count (absolute neutrophil count < 1 × 10⁹/L) or low platelet count (platelet count < 50 × 10⁹/L) at screening.
  • Being known to have HIV (human immunodeficiency virus) infection.
  • Having active hepatitis B or hepatitis C infection (viral liver disease).
  • If the investigator believes you are unwilling or unable to follow the study rules.
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Investigated drugs

NS-863 is an experimental oral medication being studied as a film‑coated tablet. In this trial, participants take the tablet by mouth to see if it can lower the pressure in the blood vessels of the lungs in people who have pulmonary hypertension linked to interstitial lung disease. The study aims to find out whether NS-863 can improve blood flow in the lungs and be safe for patients.

What is already known about the treatment

NS-863 - NS-863 is taken as a film‑coated tablet swallowed whole, usually 232 mg once a day. It is an experimental drug that is currently in Phase 2 clinical trials and has not yet been approved for regular medical use. The tablet is being studied to treat pulmonary hypertension that occurs with interstitial lung disease, a condition that raises pressure in the lung arteries. NS-863 works by blocking an enzyme called PDE‑5, which helps relax the blood vessels in the lungs and lower their resistance, and it is classified as a PDE‑5 inhibitor.

Investigated diseases

Pulmonary hypertension associated with interstitial lung disease (PH-ILD) - This condition combines high blood pressure in the arteries of the lungs with scarring or thickening of lung tissue. The increased pressure makes it harder for blood to flow through the lungs, while the scarring reduces the amount of oxygen that can be taken up. Over time, shortness of breath and fatigue become more noticeable, especially during activity. The pressure in the lung vessels often rises gradually as the lung scarring progresses, placing additional strain on the heart.
Trial detailsLast updated 18 Oct 2026
Age18+ yearsPhasePhase IITrial ID2025-525115-16-00Protocol codeNS863B-P2-01Estimated enrolment133 patientsSponsorNs Pharma Inc.

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