The study involves people with Progressive pulmonary fibrosis and Idiopathic pulmonary fibrosis, two conditions in which scar tissue builds up in the lungs and makes breathing difficult. Participants will receive an oral tablet called Admilparant (BMS‑986278), which is being tested to see how well it can be taken over a long period.
The purpose of the study is to find out whether the medication is safe and can be tolerated without serious side effects when used for an extended time. Participants will take the tablet daily and attend regular visits where simple checks are done, including blood or urine checks (lab test), a heart test that records electrical activity (ECG), and measurements of blood pressure and heart rate (vital signs) to see how the body is responding.
Throughout the study, any side effects, changes in the test results, and any serious health problems will be recorded and compared with the information collected at the start of the study. The trial is open‑label, meaning that both the researchers and participants know the medication being taken.
1baseline visit
on the day you join the study you will have a baseline visit where the study staff will record your medical history and perform initial tests.
tests include blood work, an electrocardiogram (ecg) to check heart activity, and measurements of blood pressure and heart rate (vital signs).
2receive study medication
after the baseline assessments you will be given admilparant, a medication classified as an lpa1 antagonist.
the medication is supplied as a film-coated tablet for oral use, with a dose of 9999 mg taken once each day.
3daily medication intake
you will take one tablet of admilparant each day, preferably at the same time each day, with or without food.
4regular safety visits
throughout the study you will attend scheduled visits where the same set of safety tests performed at baseline will be repeated.
these visits allow the study team to track any side effects, repeat blood work, repeat the ecg, and monitor your vital signs.
5continued medication and monitoring
you will continue taking the daily tablet for the duration of the study, which may extend for several years until the study end date or until a decision is made to stop the medication.
if a serious side effect occurs, the study staff may decide to discontinue the medication.
6study end
at the final visit the study will collect final safety data and discontinue the medication according to the protocol.
Who Can Join the Study?
You must have already finished taking the study drug and completed the final visit of the previous study (named IM027068 or IM0271015).
If you have Idiopathic pulmonary fibrosis (IPF) you need to be at least 40 years old when you sign the consent form; if you have Progressive pulmonary fibrosis (PPF) you need to be at least 21 years old.
Women who could become pregnant must use a very reliable form of birth control (one that does not depend on the user doing something each time) and must have a urine or blood pregnancy test on the first day to show they are not pregnant.
Men who have sexual contact with women who could become pregnant must agree to use condoms.
Both men and women are allowed to take part in the study.
Who Cannot Join the Study?
People who have symptoms of heart failure (the heart cannot pump blood well) while they are resting.
People who have had a lung surgery to remove part of a lung or who have received a lung transplant (a donor lung) in the past.
People who have pulmonary arterial hypertension (high blood pressure in the arteries of the lungs) and now need more than one medication to control it.
LPA1 antagonist is an oral tablet that is taken by mouth. It works by blocking a specific protein called LPA1, which is thought to be involved in the development of lung scarring in diseases such as idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). In this study, participants take the tablet for an extended period to see if it can be used safely over time without causing serious side effects. The medication is made as a film‑coated tablet and is considered an orphan drug, meaning it is intended for a rare condition.
BMS-986278 is another name for the same oral tablet that blocks the LPA1 protein. Like the LPA1 antagonist, it is taken by mouth in a film‑coated tablet form. The trial uses this medication to evaluate whether long‑term use is safe and well tolerated for people with pulmonary fibrosis. It is also classified as an orphan drug, aimed at treating a rare lung disease.
Progressive pulmonary fibrosis – It is a condition where lung tissue becomes thicker and scarred over time. The scarring makes the lungs less flexible, reducing the ability to take in oxygen. As the disease advances, breathing may become increasingly difficult, especially during activity. The process usually continues gradually, with symptoms slowly worsening. It can affect people of various ages.
Idiopathic pulmonary fibrosis – This is a type of lung scarring that occurs without a known cause. The lung tissue becomes stiff and thick, limiting the flow of air in and out of the lungs. Over months or years, the stiffness typically increases, leading to more noticeable shortness of breath. Cough may become more frequent as the disease progresses. The condition generally follows a slow, steady pattern of change.
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