AuthorisedTherapeutic exploratory (Phase II)Interventional

TIPS : Immunomodulatory Treatment of Interstitial Lung Disease Associated with Surfactant related Gene Mutations

Interstitial lung disease causes scarring and inflammation in the lungs, making breathing difficult. Some people develop this condition because of rare alterations in genes that help the lungs stay open and work properly. This study is testing whether certain medications—including steroids, azithromycin, and hydroxychloroquine—can help protect lung function. These medicines work by calming the immune system and reducing inflammation. Researchers want to find out if taking these treatments can slow down the loss of lung capacity over a 12-month period. Participants will receive medical treatment and have regular health checks, including breathing tests, scans, and heart checks. The goal is to discover safer, more effective treatments to improve quality of life and reduce the need for hospital care or lung transplants for people living with this rare condition.

At a glance

Status

Authorised

Phase

Therapeutic exploratory (Phase II)

Sponsor

Assistance Publique Hopitaux De Paris

Enrolment target

Start

14 Aug 2026

Interstitial Lung Disease

What is this study about?

The lungs contain millions of tiny air sacs that allow oxygen to enter the blood. Inside these sacs is a natural fluid called surfactant, which keeps the airways open and smooth. In some people, rare changes in their genes mean their body cannot produce or manage this fluid correctly. Over time, this causes swelling, irritation, and scarring in the lungs—a condition known as interstitial lung disease. People with this condition often experience breathlessness, a persistent cough, and fatigue.

Currently, treatment options for this specific genetic form of lung disease are limited. Doctors often try different medicines to settle the immune system, but there is not yet enough clear evidence to show which treatments work best. This study aims to fill that knowledge gap by testing specific medications—prednisolone, prednisone, azithromycin, and hydroxychloroquine—to see how well they protect lung capacity.

By comparing how patients do over the course of one year, researchers hope to see if these treatments can stabilize breathing and slow down lung damage. If successful, the study could lead to clearer guidelines for doctors and better long-term health and wellbeing for people living with this rare condition.

Key takeaways

Tests whether anti-inflammatory medicines can protect lung function in people with rare, genetic lung disease.
Open to adults aged 18 and over with confirmed surfactant-related interstitial lung disease.
Involves taking tablet medications and attending hospital check-ups over a 12-month period.
Includes regular breathing tests, chest CT scans, heart checks, and quality-of-life surveys.
Participation is entirely voluntary, and you can withdraw at any point without affecting your routine care.

Who may be eligible?

This study is open to adults aged 18 and older who have been diagnosed with interstitial lung disease linked to specific genetic changes affecting lung surfactant. Men and women can both take part.

Because this trial focuses on a very particular genetic cause, your medical team will need to confirm whether your lung disease is connected to these specific gene changes. They will also review your overall health to make sure that taking these medicines is safe for you.

People who have other severe medical conditions that make these medications unsafe, or those who cannot attend regular hospital check-ups, may not be able to participate. Your lung specialist can advise whether your specific health history fits the study requirements.

Could this study suit you?

Answer these quick questions to see if you may be eligible. This is a guide only — the research team makes the final call.

I am aged 18 or older.
I have been diagnosed with interstitial lung disease.
My lung condition is known or suspected to be linked to surfactant gene changes.
I am able to attend hospital appointments every few months for one year.
I am willing to undergo breathing tests, chest scans, and heart tracings.

Answer every question to see your result.

What does participation involve?

If you take part, your participation will last for about 12 months. You will be prescribed study medications, which may include steroid tablets (such as prednisolone or prednisone), an antibiotic called azithromycin, or a medicine called hydroxychloroquine. These are taken by mouth to help reduce inflammation.

Throughout the year, you will attend hospital visits every three months (at months 3, 6, 9, and 12). During these visits, doctors will measure how well your lungs are working using standard breathing tests called pulmonary function tests. You will also have CT scans of your chest at month 6 and month 12 to look closely at your lung tissue.

Depending on which medication you receive, you may have heart tracings (ECGs) done every few months to check your heart rhythm, as well as an eye exam near the end of the year. You will also complete short questionnaires about how you feel day-to-day and your overall quality of life.

Potential risks and benefits

Taking part may help slow down your lung condition and gives you regular, detailed health monitoring from lung experts. The knowledge gained may also help other people with this rare condition in the future. However, there is no guarantee that the treatment will directly improve your breathing. Like all medications, the study drugs can cause side effects; steroids can affect mood, sleep, or bone strength, while azithromycin and hydroxychloroquine can sometimes affect heart rhythm, stomach comfort, or vision. Your safety is monitored closely with regular heart, eye, and blood tests to spot any problems early. Taking part is completely voluntary, and you can change your mind and leave the study at any time without it affecting your standard NHS care.

Locations (1)

Some site locations are approximate. We're improving this — please verify with the trial team before travelling.

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Unverified
France

Common questions

What is interstitial lung disease?

It is an umbrella term for a group of conditions that cause inflammation and scarring (fibrosis) in the delicate tissues of the lungs, making it harder to take in oxygen.

What are surfactant gene mutations?

Surfactant is an oily liquid that coats the inside of your lungs to stop them from collapsing. Mutations are small changes in your DNA that can stop surfactant from working properly, leading to lung irritation.

Do I have to pay to take part?

No, clinical trials in the UK do not charge participants. Study medications and trial-related tests are provided free of charge, and travel expenses may sometimes be covered.

Will this cure my lung disease?

This study is not designed to cure the condition. Instead, it tests whether these medicines can slow down lung damage, ease symptoms, and protect your breathing capacity.

What happens if I experience side effects?

The research team will check on you regularly. If you develop any unpleasant side effects, doctors can adjust your dose, pause the medication, or stop it entirely to protect your health.