Immunomodulatory Treatment with Prednisone, Azithromycin and Hydroxychloroquine in Patients with Surfactant‑Related Gene Mutation Interstitial Lung Disease

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What is this study about?

The study focuses on Interstitial Lung Disease that occurs because of a change in a surfactant-related gene. The treatment being tested combines three medicines taken by mouth: prednisone (a steroid that reduces inflammation), azithromycin (an antibiotic that can also affect the immune system), and hydroxychloroquine (a drug that modifies immune activity).

The purpose of the study is to determine whether this triple therapy can slow the loss of lung function over a year. Participants will receive the medication regimen for 12 months and will return for check‑ups roughly every three months. During these visits, lung capacity will be measured with a breathing test (PFT), the chest will be imaged using a CT scan, and heart rhythm will be recorded with an ECG. An eye exam will also be performed once during the year.

Safety will be closely monitored, and any side effects will be recorded. Participants will also complete a questionnaire about overall well‑being at several points during the study, and information about hospital visits, lung transplants, and disease flare‑ups will be collected throughout the follow‑up period.

1 baseline assessment

after joining the study you will go to the clinic for the first assessment. this includes a lung function test that measures forced vital capacity (how much air you can exhale), a chest ct scan (detailed picture of the lungs), and a set of questions about your health and quality of life. you will also be asked about any current medicines and medical history.

2 start medication

you will begin taking three oral medicines every day for a total of twelve months:

prednisone 10 mg once daily – a steroid that reduces inflammation in the lungs.

azithromycin 250 mg once daily – an antibiotic that may help modify the immune response.

hydroxychloroquine 400 mg once daily – a drug that can affect the immune system.

the study staff will give you instructions on how to take each pill and will provide a calendar to track your doses.

3 3‑month visit

three months after starting the medicines you will return to the clinic.

you will complete a quality of life questionnaire that asks about symptoms, daily activities, and overall well‑being.

an ecg (electrocardiogram – a simple test that records the electrical activity of the heart) will be performed because hydroxychloroquine and azithromycin can affect the heart rhythm.

the staff will ask you to report any side effects such as stomach upset, skin changes, or vision problems.

4 6‑month visit

six months after enrollment you will have another clinic visit.

a repeat lung function test will be done to measure forced vital capacity again.

a follow‑up chest ct scan will be performed to look at changes in the lungs.

you will fill out the same quality of life questionnaire as before.

another ecg will be recorded to monitor heart rhythm.

any new or worsening side effects should be reported to the study team.

5 9‑month visit

at nine months you will attend a routine visit.

you will again answer the quality of life questionnaire and undergo an ecg to check heart rhythm.

the staff will review your medication adherence and ask about any adverse symptoms.

6 12‑month visit

one year after starting treatment you will complete the final study visit.

the visit includes a lung function test measuring forced vital capacity, a chest ct scan, and the quality of life questionnaire.

an ecg will be performed, and an ophthalmological exam (eye exam) will be done to detect any eye changes that can be caused by hydroxychloroquine.

the study team will collect information about any hospitalizations, lung transplants, or disease worsening that occurred during the year.

Who Can Join the Study?

Age 18 years or older but younger than 80 years.
Must have a genetic change (called a variant) that experts have judged to be disease‑causing (pathogenic) or probably disease‑causing, in one of these genes: SFTPA1, SFTPA2, SFTPC, NKX2‑1, or SFTPB; or must have two such variants in the ABCA3 or SFTPB genes. This judgment is made by a team of genetic specialists (a genetic multidisciplinary discussion).
Must have interstitial lung disease (a condition where the tissue around the air sacs of the lungs becomes scarred) that involves more than 10 % of the total lung volume on a CT scan (a detailed X‑ray picture) taken within the past 2 years.

Who Cannot Join the Study?

Contraindication to azithromycin, hydroxychloroquine or prednisone – this means you have a medical reason that makes it unsafe for you to take any of these medicines (azithromycin is an antibiotic, hydroxychloroquine affects the immune system, and prednisone is a steroid that reduces inflammation).
Pregnancy or breastfeeding – if you are currently pregnant or feeding a baby, you cannot join because the study drugs might affect the baby.
Participation in another interventional study (a clinical trial that tests a medicinal product, medical device, or any other experimental treatment involving people) – you may only be in one study at a time.
Being deprived of liberty (for example, in prison) or under a legal protection measure (such as a court‑ordered guardianship) – these situations prevent participation.
Lack of any health insurance system affiliation – you must have health insurance coverage to be eligible.

Where you can join this trial?

Verified and Recommended Sites

No sites found in this category

Verified Sites

Site Name	City	Country	Status
Centre Hospitalier Universitaire De Lille	Lille	France

Other Sites

Site Name	City	Country
Aughnhyczn Pbmozhuc Hhdhjzbl Dz Psung	Paris	France
Czaqjv Hrgxmgfrqdg Ufywfzfabrufb Da Rqvfrz	Rennes	France
Hemaaqmd Cqfvrf Dy Lkwn	Bron	France

Trial status

Country	Status	Recruitment Start
France	Not yet recruiting	01.09.2026

Trial locations

Investigated Drugs:

Prednisone is a medicine taken by mouth that reduces inflammation and suppresses the immune system. In this study it is used to calm the over‑active immune response that can damage the lungs in people with interstitial lung disease linked to surfactant‑related gene changes.

Azithromycin is an oral antibiotic that also has effects on the immune system. In the trial it is given to help control any hidden infections and to provide additional anti‑inflammatory benefits that may improve lung function.

Hydroxychloroquine is a pill that was originally used for malaria but is now known to modify the immune system. In this research it is added to the treatment plan to further reduce abnormal immune activity that contributes to lung scarring.

Investigated Diseases:

Interstitial lung disease

Interstitial Lung Disease – Interstitial Lung Disease is a group of disorders that cause scarring and inflammation in the tissue surrounding the tiny air sacs of the lungs. The scarring makes the lung tissue stiff, reducing its ability to expand and absorb oxygen. Over time, the disease can cause a gradual decline in breathing capacity and may lead to persistent cough and shortness of breath. In cases linked to a surfactant‑related gene variant, the abnormal surfactant can trigger early inflammation and faster accumulation of scar tissue. The condition typically progresses slowly but can accelerate, leading to worsening lung function over months to years.

Trial ID:

2026-525336-41-00

NCT ID:

NCT07443436

Trial Phase:

Therapeutic exploratory (Phase II)

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Immunomodulatory Treatment with Prednisone, Azithromycin and Hydroxychloroquine in Patients with Surfactant‑Related Gene Mutation Interstitial Lung Disease

What is this study about?

Who Can Join the Study?

Who Cannot Join the Study?