If you have idiopathic pulmonary fibrosis (IPF), a chronic lung condition that can cause
shortness of breath, low oxygen levels in the blood, and scarring on the lungs (fibrosis), it’s important to know that treatment is available to help slow the disease’s progression, manage symptoms, and improve your quality of life.
“IPF treatment starts as soon as the diagnosis is confirmed,” says Kenneth D’Souza, MD, a pulmonologist and the medical director of the intensive care unit at Mercy Health in Cincinnati. Treatment options include medications, surgery, lifestyle changes, rehabilitation, and therapy.
Your healthcare team will consider how severe the disease is, how quickly it’s progressing, your symptoms, and your overall health when recommending a treatment plan, Dr. D’Souza adds. Talk to your doctor before starting any treatments to discuss which options are best for you.
Different types of medications are often part of an IPF treatment plan. Some are used to slow the progression of fibrosis, while others help relieve symptoms.
Antifibrotics
Antifibrotics are the primary medications used to treat IPF, and there are currently three options approved by the U.S. Food and Drug Administration (FDA), says
Guillermo Garrido, MD, a board-certified physician with Orlando Health Medical Group Pulmonary and Sleep Medicine, based in Orlando, Florida, with a special interest in advanced and interstitial lung disease.
Rather than reversing the scarring that has already occurred, antifibrotics help slow the rate at which the lungs build scar tissue and help preserve lung function over time, says D’Souza. Because of this, they’re typically started at or soon after diagnosis, depending on the individual, he adds.
Antifibrotic medications used to treat IPF include:
- nintedanib (Ofev)
- pirfenidone (Esbriet)
- nerandomilast (Jascayd)
All three are taken by mouth. Nerandomilast received FDA approval in 2025, making it the first new treatment approved for IPF in more than a decade.
It works through a different mechanism than the other two antifibrotics and is generally used as an alternative for people who are not able to tolerate nintedanib or pirfenidone, says D’Souza.
Side effects of these medications vary, but they may include:
- Diarrhea
- Nausea or vomiting
- Loss of appetite or weight loss
- Elevated liver enzymes
- Fatigue
- Headache or dizziness
Some people may experience sun sensitivity or a skin rash with pirfenidone, so protecting your skin from sun exposure is important.
Your healthcare team will monitor you for side effects and may adjust your dose or treatment if needed.
Corticosteroids
Corticosteroids (commonly referred to simply as steroids) may be prescribed during flare-ups, also called acute exacerbations, says D’Souza. These medications help reduce lung inflammation and may relieve symptoms during these episodes.
There’s limited evidence on how much they improve outcomes, so they’re generally reserved for short-term use.
Steroid medications used in IPF include prednisone (Deltasone, Predone, Sterapred) and methylprednisolone (Medrol).
Common side effects may include:
- Trouble sleeping
- Weight gain
- Upset stomach
- Insomnia
- Swelling in the legs and midsection
- High blood pressure
- High blood sugar levels
- Mood changes, such as irritability or anxiety
- Increased risk of infection
Acid Reflux Medications
Many people with IPF also have
gastroesophageal reflux disease (GERD), or acid reflux.
When stomach acid flows back up into the esophagus, small amounts can sometimes reach the airways or be inhaled into the lungs. This can irritate lung tissue and may worsen symptoms like coughing or shortness of breath.
Medications used to treat GERD work by either blocking acid production in the stomach or reducing how much acid is released.
While research is still ongoing, these medications are used to manage GERD symptoms rather than treat IPF.
Acid reflux medications that may be used include:
- omeprazole (Prilosec)
- esomeprazole (Nexium)
- famotidine (Pepcid)
Side effects of these medications may include:
- Headache
- Nausea or upset stomach
- Diarrhea or constipation
Cough Suppressants
A persistent, dry cough is a common and often frustrating symptom of IPF, and it can be difficult to control with standard cough medications.
Your doctor may recommend a cough suppressant to help, though what works varies from person to person, says D’Souza.
Depending on your symptoms, your healthcare team may recommend over-the-counter or prescription options.
Cough suppressants that may be used in IPF include dextromethorphan (Delsym, Robitussin) and benzonatate (Tessalon Perles).
A lung
transplant is currently the only surgical treatment for IPF. In this procedure, one or both diseased lungs are replaced with healthy donor lungs.
It’s a major surgery, and there are risks to consider, including infection and the body rejecting the new lungs.
After a lung transplant, lifelong medication is needed.
“Guidelines recommend transplant evaluation as soon as an [IPF] diagnosis is made,” Dr. Garrido says. Early evaluation can give you time to understand the risks, benefits, and how a transplant might affect your quality of life, he adds.
A lung transplant evaluation often includes a review of your:
- Overall physical health and fitness
- Heart, kidney, and liver function
- Bone health
- Mental and emotional readiness
- Social support and ability to manage care after surgery
This type of surgery isn’t the right option for everyone with IPF. Lung transplant candidates are typically younger than 70 — though that’s flexible — and generally in good health outside of their lung disease, says D’Souza.
For some people, a lung transplant may offer more time and an improved quality of life. For others, the risks may outweigh the benefits.
You can discuss these factors with your care team as you consider your options.
Lifestyle adjustments can’t stop or reverse IPF, but they can help you manage symptoms and feel your best. Experts recommend the following to help protect your lung health:
- Quit smoking. This includes cigarettes, vaping, marijuana, and other inhaled irritants.
- Stay up to date on vaccinations. Flu and pneumonia vaccines can help lower your risk of respiratory infections.
- Stay active. Regular movement, including walking or light exercise, can help improve stamina and reduce shortness of breath.
- Maintain a healthy weight. Weight loss, if you’re carrying extra weight, may help you breathe more comfortably and improve mobility.
- Eat a balanced diet. Good nutrition helps support energy levels and overall health.
- Limit exposure to lung irritants. Avoid dust, fumes, pollution, and other triggers.
- Manage other health conditions. If you have other health conditions like sleep apnea, diabetes, or heart disease, managing those well is an important part of your care.
Some IPF therapies can help make breathing easier and improve your overall well-being, though they don’t treat underlying scarring of the lungs.
Oxygen Therapy
Supplemental oxygen may be recommended if your oxygen levels drop below 88 percent either at rest or during activity, says D’Souza. This is often determined using a six-minute walk test, in which you walk for six minutes while your oxygen levels and breathing are monitored, says D’Souza.
For some people, supplemental oxygen is only needed during sleep or physical activity. Others may rely on it more regularly. Portable oxygen systems can help you stay mobile and maintain your daily activities.
Pulmonary Rehabilitation
Pulmonary rehab is a supervised program that combines exercise, breathing techniques, and education about your condition.
D’Souza notes that this program can increase your ability to stay physically active and help you feel less short of breath.
Because a qualified respiratory therapist and other healthcare professionals monitor you during these sessions, you can exercise safely at an intensity that works for you.
Almost anyone whose quality of life has been impacted by IPF can benefit from pulmonary rehab, says Garrido.
It’s also a great way to improve your overall physical readiness if you are considering a lung transplant, he adds.
It’s common to experience feelings of
anxiety or depression when you’re living with a chronic condition like IPF.
The changes in your mobility and the effort required to manage your symptoms can affect your mood, and acknowledging those feelings is an important part of your care.
Talk with your doctor about whether working with a mental health professional might be helpful.
Mindfulness meditation and yoga may also help you cope with anxiety, stress, and shortness of breath related to the disease, says Garrido. D’Souza suggests breathing exercises and support groups as helpful approaches.
Clinical trials for IPF are underway through organizations like the Pulmonary Fibrosis Foundation, the National Institutes of Health, and major medical centers, says D’Souza.
New medications that target different parts of the scarring process are being studied, including inhaled therapies and drugs that may be combined with existing treatments, he adds.
There has already been meaningful progress in the treatment of IPF, and Garrido anticipates more innovations in the coming years.
If you’re interested in participating in a clinical trial, talk with your care team about available options. You can also search for currently recruiting studies through the Pulmonary Fibrosis Foundation’s Clinical Trials Resource Center and ClinicalTrials.gov.
