Idiopathic pulmonary fibrosis is a chronic lung disease that causes progressive scarring of lung tissue, making breathing increasingly difficult. While there is currently no cure, understanding treatment approaches—both established and experimental—can help patients and their families navigate this challenging diagnosis and make informed decisions about care.

Understanding How Treatment Helps Manage Lung Scarring

When someone receives a diagnosis of idiopathic pulmonary fibrosis, often shortened to IPF, the first question is usually about what can be done. The main goals of treatment focus on slowing down the progression of lung scarring, managing symptoms like shortness of breath and cough, and improving quality of life for as long as possible.^[1] Treatment decisions depend heavily on how advanced the disease is, how quickly it appears to be progressing, and the overall health of the patient.^[3]

Medical professionals recommend a combination of approaches tailored to each person’s situation. These include medications approved by health authorities around the world, supportive therapies to make breathing easier, lifestyle changes, and in some cases, consideration of lung transplantation.^[4] It’s important to understand that IPF affects each person differently—some people experience slow progression over many years, while others may see their condition worsen more quickly.^[1]

Beyond standard treatments available today, researchers are actively investigating new therapies through clinical trials. These studies test innovative approaches that may offer additional options in the future. Patients who are interested can discuss with their doctors whether participating in research might be appropriate for their situation.^[13]

Standard Medical Treatments Currently Available

Two main medications have been approved specifically to treat idiopathic pulmonary fibrosis. These drugs, known as antifibrotic medications (meaning they work against the scarring process), represent the cornerstone of modern IPF treatment. They cannot reverse damage already done, but clinical studies have shown they can slow down the rate at which lung function declines.^[10]

Pirfenidone

Pirfenidone works by reducing the activity of the immune system and slowing the scarring process in the lungs. It is taken as capsules three times daily with food.^[10] Clinical trials have demonstrated that pirfenidone can slow the progression of mild to moderate IPF, helping to preserve lung capacity over time.^[15] The medication is recommended for patients whose lung function tests show their capacity is between 50% and 80% of what would normally be expected for their age and size.^[10]

Like any medication, pirfenidone can cause side effects. Common ones include feeling sick to the stomach, tiredness, diarrhea, indigestion, and sensitivity to sunlight that can cause skin rashes.^[10] Patients taking this medication need regular blood tests to monitor liver function, as there have been rare reports of liver damage. Warning signs to watch for include yellowing of the eyes or skin, loss of appetite, itchy skin, darker urine, unusual tiredness, or pain in the upper right part of the stomach.^[10] If any of these symptoms appear, it’s crucial to stop the medicine and contact a doctor immediately.

Nintedanib

Nintedanib represents a different approach to fighting lung scarring. This medication is a tyrosine kinase inhibitor, which means it blocks certain enzymes involved in the scarring process. It was originally developed as an anticancer treatment before researchers discovered it could slow fibrosis by inhibiting growth factors that promote scarring, including vascular endothelial growth factor, platelet-derived growth factor, fibroblast growth factor, and transforming growth factor.^[14]

Nintedanib is taken as capsules twice daily with food.^[15] Like pirfenidone, it has been shown in clinical trials to slow the decline in lung function for people with IPF. It is approved not only for idiopathic pulmonary fibrosis but also for certain other forms of progressive lung scarring, including scleroderma-associated interstitial lung disease.^[15]

The most common side effect of nintedanib is diarrhea, which can be bothersome but in most cases does not require stopping the medication.^[14] Other possible side effects include nausea, vomiting, abdominal pain, and loss of appetite leading to weight loss.^[10] Similar to pirfenidone, nintedanib is typically recommended when lung function tests show capacity between 50% and 80% of expected, and should be stopped if lung capacity drops significantly despite treatment.^[10]

Supportive Treatments That Make Daily Life Easier

Beyond medications that target the scarring process itself, several supportive treatments help manage symptoms and maintain quality of life. These are not optional extras—they are essential parts of comprehensive IPF care.

Supplemental oxygen therapy becomes necessary when the scarred lungs can no longer deliver enough oxygen to the bloodstream. When blood oxygen levels fall, patients feel more breathless and tire easily. Oxygen delivered through nasal tubes or a mask connected to a concentrator machine helps improve breathing and allows people to be more active.^[10] The tubes are long enough to allow movement around the home, and portable oxygen devices are available for use outside the house.^[10]

Pulmonary rehabilitation is a structured program that combines education, exercise, breathing techniques, nutritional advice, psychological support, and connection with others facing similar challenges.^[10] These programs help people understand their condition better, learn to manage breathlessness, stay as physically active as possible, and maintain independence in daily activities. Rehabilitation courses are typically held locally and tailored to individual capabilities.^[10]

Lifestyle Changes and Self-Care

Healthcare professionals strongly encourage several lifestyle measures that help protect lung health. Patients who smoke must stop immediately and should be offered help to quit, as smoking accelerates lung damage.^[4] Regular exercise within individual limits, eating a balanced and healthy diet, and staying as physically fit as possible all contribute to better outcomes.^[4]

Vaccination is another critical protective measure. People with IPF should receive the annual flu vaccine and the pneumococcal vaccine, because lung infections can be particularly serious and even life-threatening when lungs are already compromised.^[4] Avoiding close contact with people who have colds or chest infections whenever possible also reduces risk.^[4]

Lung Transplantation

For some patients with advanced IPF, lung transplantation may be considered. This is a major surgical procedure where one or both damaged lungs are replaced with healthy lungs from a donor. Transplantation is the only treatment shown to actually extend life expectancy in IPF.^[13] However, it is not suitable for everyone.

The decision about whether to pursue transplant depends on multiple factors: how severe the disease is, how quickly it is progressing, the patient’s age and overall health, how much improvement could reasonably be expected after transplant, and most importantly, whether a suitable donor lung is available.^[10] The waiting time for donor organs can be long, and the surgery itself carries significant risks. After transplant, patients need lifelong medications to prevent rejection and require close medical monitoring.^[10]

Promising Treatments Being Tested in Clinical Trials

While pirfenidone and nintedanib represent important advances, they only slow disease progression—they don’t stop it entirely or reverse damage. This has driven researchers worldwide to investigate new therapeutic approaches through clinical trials. These studies are essential for finding better treatments and potentially, one day, a cure.

Understanding Clinical Trial Phases

Before discussing specific experimental therapies, it helps to understand how clinical trials work. New treatments progress through three main phases. Phase I trials involve small numbers of participants and focus primarily on safety—determining what doses are safe and what side effects occur. Phase II trials include more participants and aim to determine whether the treatment shows efficacy (whether it works) and to gather more safety information. Phase III trials are large studies that compare the new treatment directly against current standard treatments or placebo to confirm effectiveness and monitor side effects in diverse populations.^[13]

Novel Anti-Fibrotic Approaches

Researchers are investigating several new molecules that target different pathways involved in lung scarring. One such approach involves phosphodiesterase inhibitors. A drug called nerandomilast has been studied in clinical trials for IPF. This medication works by modulating inflammation and potentially reducing fibrosis through a different mechanism than existing treatments.^[8]

The advantage of drugs that work through different mechanisms is that they might be combined with existing treatments like pirfenidone or nintedanib to provide additive benefits. However, such combinations require careful study to ensure they are safe when used together.

Targeting Specific Molecular Pathways

Recent research has identified specific molecules and cellular processes that drive the scarring process in IPF. For example, scientists at Vanderbilt University Medical Center and the University of Michigan Medical School recently published research identifying a potential new treatment target. Their laboratory studies, published in early 2025, explored ways to address the progressive accumulation of scar tissue in the airways.^[16]

While this research is still in early stages and involves laboratory work rather than human trials, it represents the type of targeted approach that may lead to future therapies. By understanding the precise molecular events that cause lung cells to produce excessive scar tissue, researchers can design drugs that specifically interrupt those processes.

Immunotherapy and Inflammation Control

Some forms of lung scarring involve immune system dysfunction and inflammation. Although IPF is not primarily an inflammatory disease like some other lung conditions, researchers are investigating whether certain immunotherapies might help. For instance, tocilizumab, a medication that blocks a specific inflammatory molecule, has been studied in certain types of progressive lung fibrosis. This drug interrupts inflammatory processes and has shown promise in reducing the rate of lung function decline in specific patient populations.^[15]

Repurposing Existing Drugs

Sometimes, medications developed for other conditions show promise for treating IPF. This is called drug repurposing. While it once appeared that drugs to reduce stomach acid might help IPF patients, newer research and updated guidelines do not support using antacid therapy specifically to improve lung function or slow disease progression.^[14] This illustrates how clinical research continuously refines our understanding of what works and what doesn’t.

Finding and Joining Clinical Trials

Patients interested in clinical trials should discuss this option with their pulmonologist or IPF specialist. Trials are conducted at specialized centers, often at university hospitals or research institutions, in locations including the United States, Europe, and other regions around the world.^[7] Eligibility criteria vary depending on the specific study—some trials seek newly diagnosed patients, while others look for people with more advanced disease or those who haven’t responded well to standard treatments.

Participating in research contributes to advancing knowledge that will benefit future IPF patients. However, it’s important to understand that experimental treatments may not work, may cause unexpected side effects, and often involve more frequent monitoring visits than standard care. Clinical trial teams provide detailed information about what participation involves, and patients have the right to withdraw at any time.

Managing Complications and Acute Worsening

Even with treatment, IPF can lead to complications that require additional medical attention. One serious complication is pulmonary hypertension, which means high blood pressure in the arteries of the lungs. This occurs when scarred, stiff lung tissue makes it harder for blood to flow through the lungs, forcing the heart to work harder.^[7] Another potential complication is respiratory failure, which happens when the lungs become so damaged they cannot deliver enough oxygen to the body even with supplemental oxygen support.^[1]

Many people with IPF experience what are called acute exacerbations—sudden worsening episodes where symptoms become dramatically worse over days or weeks. These events can occur at any time, sometimes triggered by infections or other factors, but often with no obvious cause.^[1] Exacerbations are medical emergencies that require immediate attention. Treatment typically involves corticosteroid medications to reduce inflammation, along with supplemental oxygen and supportive care.^[14] Unfortunately, acute exacerbations can be life-threatening and often indicate more advanced disease.^[19]

The Importance of Comprehensive Care Teams

Managing IPF effectively requires coordination among multiple healthcare professionals. Beyond the pulmonologist who specializes in lung diseases, patients may also see cardiologists (heart doctors), nutritionists, respiratory therapists, physical therapists, and mental health professionals. This team approach ensures that all aspects of the disease and its impact on daily life are addressed.^[9]

Regular monitoring is essential. Follow-up visits typically include breathing tests to measure lung function, imaging scans to assess scarring progression, and checks for complications like low oxygen levels or pulmonary hypertension.^[19] These assessments help doctors adjust treatment plans as needed and make timely decisions about whether to consider options like transplant evaluation.

Most Common Treatment Methods

• Antifibrotic Medications
  • Pirfenidone taken three times daily as capsules to slow lung scarring by reducing immune system activity
  • Nintedanib taken twice daily as capsules to block enzymes involved in fibrosis formation
  • Both medications slow decline in lung function but cannot reverse existing damage
  • Recommended when lung capacity measures 50-80% of expected normal values
• Oxygen Therapy
  • Supplemental oxygen delivered through nasal tubes or masks when blood oxygen levels fall
  • Home oxygen concentrators with long tubing for mobility
  • Portable oxygen devices for use outside the home
  • Helps reduce breathlessness and allows increased activity
• Pulmonary Rehabilitation
  • Structured programs combining exercise training tailored to individual capacity
  • Breathing techniques to manage shortness of breath
  • Education about the disease and self-management strategies
  • Nutritional counseling and psychological support
  • Connection with support groups and others living with similar conditions
• Lung Transplantation
  • Single or double lung transplant surgery replacing damaged lungs with healthy donor organs
  • Only treatment proven to extend life expectancy in advanced IPF
  • Requires careful patient selection based on disease severity, progression rate, age, and overall health
  • Limited by availability of suitable donor organs
  • Requires lifelong immunosuppressive medications after surgery
• Symptom Management
  • Cough suppressants and mucus-thinning medications to manage persistent cough
  • Treatment for gastroesophageal reflux when present
  • Management of anxiety and depression which commonly accompany chronic lung disease
  • Palliative care services for advanced disease to maximize comfort and quality of life
• Preventive Measures
  • Annual influenza vaccination to prevent respiratory infections
  • Pneumococcal vaccination to protect against pneumonia
  • Smoking cessation support and programs
  • Avoiding exposure to people with respiratory infections