Pulmonary Fibrosis Medications: What You Need to Know

TL;DR

- Pulmonary fibrosis (PF) cannot be cured, but two FDA-approved antifibrotic medications nintedanib (Ofev) and pirfenidone (Esbriet), can slow the progression of scarring in the lungs. Neither reverses damage, but both have been shown in clinical trials to reduce the rate of lung function decline.

- Additional medications manage symptoms like cough, breathlessness, and acid reflux, which often make PF worse.

- Working closely with a pulmonologist who specializes in interstitial lung diseases gives you the best chance of finding the right combination for your situation.

What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a lung disease where scar tissue (fibrosis) builds up inside the lungs over time. This scarring makes the lungs stiffer and less able to move oxygen into the bloodstream. The most common form, idiopathic pulmonary fibrosis (IPF), has no known single cause.

Symptoms include shortness of breath, a dry persistent cough, fatigue, and reduced ability to exercise. Because PF progresses differently in each person, treatment plans are highly individual.

Can Medications Cure Pulmonary Fibrosis?

No medication currently available can cure pulmonary fibrosis or reverse existing lung scarring. However, two antifibrotic drugs have strong clinical evidence showing they slow the rate of decline in lung function. For many patients, this means more time with better breathing capacity and quality of life.

The Two Main Antifibrotic Medications

What Is Nintedanib (Ofev)?

Nintedanib is an oral capsule taken twice daily with food. It works by blocking proteins that signal the body to produce scar tissue in the lungs. A major clinical trial (INPULSIS) found that nintedanib reduced the annual rate of lung function decline by approximately 50% compared to placebo.

Common side effects include diarrhea, nausea, and stomach pain. These are most common in the first few months and often improve with dose adjustments or anti-diarrheal medication. Liver enzyme levels should be monitored regularly.

What Is Pirfenidone (Esbriet)?

Pirfenidone is an oral tablet taken three times daily with food. It has anti-inflammatory and antifibrotic properties, reducing the activity of proteins that drive lung scarring. The ASCEND and CAPACITY trials showed pirfenidone significantly slowed disease progression in IPF patients.

Common side effects include nausea, fatigue, skin sensitivity to sunlight (photosensitivity), and rash. Wearing sunscreen and protective clothing outdoors is important for patients taking pirfenidone. Liver function should also be monitored.

How Do Doctors Choose Between Nintedanib and Pirfenidone?

There is no definitive head-to-head trial proving one drug is better than the other. Doctors typically consider:

• Your other health conditions: nintedanib may be preferred in patients with certain cardiovascular risks; pirfenidone may suit those prone to gastrointestinal issues less well
• Side effect tolerance: diarrhea is more associated with nintedanib; sun sensitivity and skin issues are more common with pirfenidone
• Your daily routine: pirfenidone requires three doses per day versus two for nintedanib
• Cost and insurance coverage: both drugs are expensive; your care team can help navigate patient assistance programs

Some patients switch between the two if side effects are unmanageable. Others try one and stay on it for years.

Medications That Help Manage Symptoms

Beyond antifibrotic drugs, several medications address the symptoms of PF that affect daily life.

Acid Reflux (GERD) Treatment: Research suggests that acid reflux may worsen PF by causing micro-aspiration of stomach acid into the lungs. Proton pump inhibitors (PPIs) such as omeprazole are often prescribed even in patients without obvious reflux symptoms.

Cough Suppressants: Chronic dry cough is one of the most distressing symptoms of PF. Low-dose morphine or codeine is sometimes used for severe cough. Newer drugs targeting the cough reflex are being studied in clinical trials.

Oxygen Therapy: Supplemental oxygen is not a medication in the traditional sense, but it is a critical treatment when blood oxygen levels drop, especially during activity or sleep. Oxygen improves exercise tolerance and quality of life.

Pulmonary Rehabilitation: Exercise programs designed for lung disease patients help strengthen breathing muscles and improve endurance. Studies show pulmonary rehabilitation meaningfully improves how patients feel day to day.

What About Steroids and Immunosuppressants?

For many years, corticosteroids (like prednisone) and immunosuppressant drugs were commonly prescribed for IPF. A landmark study called PANTHER-IPF found that a combination of prednisone, azathioprine, and N-acetylcysteine actually increased hospitalizations and deaths in IPF patients compared to placebo. As a result, this combination is no longer recommended for IPF.

However, steroids and immunosuppressants are used in other forms of interstitial lung disease (ILD) where inflammation plays a larger role, such as hypersensitivity pneumonitis or connective tissue disease–associated ILD. Your doctor's recommendations will depend on your specific diagnosis.

What About Lung Transplant?

For eligible patients, typically younger individuals with severe or rapidly progressing disease, lung transplantation offers the possibility of significantly improved survival and quality of life. Antifibrotic medications may be used to slow decline while a patient waits for a transplant. Transplantation is not suitable for everyone, and the evaluation process is thorough. Discussing this option early with your specialist is worthwhile.

New and Emerging Treatments

Research into pulmonary fibrosis is active. Several promising treatments are in late-stage clinical trials as of 2025, including:

• Inhaled treprostinil
• Monoclonal antibodies targeting specific fibrotic pathways (e.g., pamrevlumab, bexotegrast)
• Combination therapy trials testing whether nintedanib plus an additional agent provides added benefit

Enrolling in a clinical trial may give you access to emerging therapies. Ask your healthcare provider to help you with the process.

Managing Side Effects: Practical Tips

Starting a new antifibrotic medication can feel overwhelming. These strategies help many patients manage the early adjustment period:

• Take medication with food: both nintedanib and pirfenidone cause fewer stomach problems when taken with a meal
• Stay hydrated: especially important if experiencing diarrhea with nintedanib
• Use sunscreen daily: essential for pirfenidone users; SPF 30 or higher on all exposed skin
• Keep a symptom diary: noting when side effects occur helps your doctor adjust your dose or timing
• Don't stop suddenly: if side effects are difficult, contact your doctor before stopping. Dose reduction is often preferable to stopping entirely

Pulmonary fibrosis is a serious condition, but treatment options have improved significantly over the past decade. Antifibrotic medications slow lung function decline, symptom management improves daily comfort, and ongoing research continues to expand what is possible. Staying engaged with a specialist team and advocating for yourself makes a real difference in outcomes.

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Disclaimer:

This article is for informational purposes only and does not replace advice from a qualified medical professional. Always consult your doctor before starting, stopping, or changing any medication.

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