There is no single best treatment for pulmonary fibrosis, but a combination of antifibrotic medications, pulmonary rehabilitation, supplemental oxygen, and in advanced cases, lung transplantation offers the strongest approach to slowing the disease and maintaining quality of life. Pulmonary fibrosis has no cure, so treatment focuses on slowing lung scarring, managing symptoms, and preserving the ability to stay active as long as possible.
Antifibrotic Medications
Two antifibrotic drugs have been the standard of care for idiopathic pulmonary fibrosis (IPF) for more than a decade: pirfenidone and nintedanib. Both work by interfering with the biological signals that drive scar tissue formation in the lungs. Specifically, they block the activity of growth factors that tell cells to produce excess collagen, the protein that makes up scar tissue. They also appear to directly inhibit collagen fibers from assembling outside of cells, which is a distinct way of putting the brakes on scarring at multiple stages.
Neither drug reverses existing damage. What they do is slow the rate at which lung function declines, typically measured by how much air the lungs can hold and push out. For many people, this translates into more time before the disease reaches an advanced stage. The 2022 international clinical practice guideline from the American Thoracic Society and partner organizations gives a conditional recommendation for nintedanib in progressive pulmonary fibrosis and recommends additional research into pirfenidone for the same population.
In October 2025, the FDA approved a new medication called nerandomilast (brand name Jascayd) for IPF. This was the first new therapy approved for the condition in over 10 years, expanding the options beyond pirfenidone and nintedanib.
Side Effects of Antifibrotic Drugs
Gastrointestinal problems are the most common side effects of both medications, though they show up differently. Nintedanib causes diarrhea in roughly 60% of people who take it, along with nausea in about 22 to 26%. The diarrhea can be significant enough that some people need a dose reduction. Liver function should be monitored regularly, and if liver-related problems arise, lowering the dose or stopping the drug typically reverses them.
Pirfenidone’s gastrointestinal profile leans more toward nausea (about 36% of people) and indigestion (about 19%). Its most distinctive side effect, though, is photosensitivity. Severe sunburn-like reactions are common enough that anyone taking pirfenidone needs to take sun protection seriously: high-SPF sunscreen, long sleeves, and avoiding prolonged time in direct sunlight, especially during peak hours. These skin reactions can be intense but resolve once exposure is managed.
Side effects are a real factor in choosing between these drugs. Your doctor will likely consider your digestive health, sun exposure habits, and liver function when recommending one over the other. Many people tolerate these medications well enough to stay on them long term, and managing side effects early makes a significant difference in sticking with treatment.
Pulmonary Rehabilitation
Pulmonary rehabilitation is one of the most effective non-drug treatments for pulmonary fibrosis, and it is often underutilized. It typically involves supervised exercise training, breathing techniques, and education about managing the disease. The benefits are well documented across multiple studies: people who complete a rehabilitation program walk farther, feel less short of breath, and report better quality of life compared to those who don’t participate.
In one study, participants improved their six-minute walk distance by an average of about 46 meters after a 10-week exercise program. Another found an 81-meter improvement compared to a control group. That may not sound dramatic on paper, but for someone with progressive lung scarring, walking an extra city block without stopping is a meaningful change in daily life. Quality of life scores improved as well, with gains in both physical function and mental health that persisted for at least three months after the program ended.
Rehabilitation also appears to complement antifibrotic medications. One study found that patients receiving both pulmonary rehabilitation and antifibrotic drugs improved their walk distance by about 34 meters, while those on medication alone declined by roughly the same amount. The combination was clearly more effective than either approach on its own.
Supplemental Oxygen
As pulmonary fibrosis progresses, the lungs become less efficient at transferring oxygen into the bloodstream. Supplemental oxygen doesn’t treat the scarring, but it relieves breathlessness, supports physical activity, and protects the heart from the strain of chronically low oxygen levels.
Current American Thoracic Society guidelines recommend long-term oxygen therapy for at least 15 hours per day when resting blood oxygen saturation drops to 88% or below. Some people initially need oxygen only during exercise or sleep, then transition to using it more throughout the day as the disease advances. Portable oxygen concentrators have made it easier to stay active while using supplemental oxygen, which matters because staying physically active is one of the best things you can do for your overall health with this condition.
Lung Transplantation
For people whose disease continues to worsen despite medication and rehabilitation, lung transplantation is the only treatment that can significantly extend life. IPF is now one of the leading reasons for lung transplants worldwide. The key is early referral: international guidelines recommend that transplant evaluation begin as soon as certain markers appear, not when the disease has reached its final stages.
Referral to a transplant center is recommended when lung capacity (measured as forced vital capacity) drops below 40% of the predicted value, when you need any amount of supplemental oxygen, or when shortness of breath begins limiting daily activities. The threshold for being placed on the active transplant waiting list is higher: a decline in lung capacity of 10% or more over six months, oxygen saturation dropping below 88%, a six-minute walk distance under 250 meters, development of pulmonary hypertension, or hospitalization for a respiratory flare.
Transplant evaluation is a lengthy process involving medical, psychological, and logistical assessments. Not everyone is a candidate, and age, other health conditions, and the availability of donor organs all factor in. But for those who do receive a transplant, the improvement in breathing and quality of life can be dramatic.
The Role of Acid Reflux Treatment
Gastroesophageal reflux disease (GERD) is extremely common in people with pulmonary fibrosis, and there has been longstanding debate about whether treating it slows lung damage. The theory is that tiny amounts of stomach acid aspirated into the lungs could worsen scarring. Earlier guidelines conditionally recommended proton pump inhibitors (PPIs) based on data suggesting they could stabilize lung function and reduce disease flares.
More recent evidence has complicated this picture. Some studies have linked PPI use to an increased risk of lung infections and potentially worse outcomes. The 2022 international guidelines reversed course, issuing a conditional recommendation against antacid medication and antireflux surgery specifically for treating IPF. If you have GERD symptoms that affect your comfort, treating them still makes sense, but the idea that acid reflux treatment slows pulmonary fibrosis itself remains unproven.
Putting a Treatment Plan Together
The most effective approach to pulmonary fibrosis combines several of these strategies rather than relying on any single one. A typical treatment plan for someone with IPF includes an antifibrotic medication started as early as possible, enrollment in a pulmonary rehabilitation program, supplemental oxygen when blood oxygen levels warrant it, and early discussion about transplant eligibility for those with progressive disease. Vaccinations against respiratory infections, including influenza and pneumonia, are also standard because any lung infection can trigger a dangerous flare in someone with existing scarring.
The pace of progression varies enormously from person to person. Some people remain stable for years on antifibrotic therapy, while others decline more rapidly despite treatment. Regular monitoring of lung function, typically every three to six months, helps your care team adjust the plan as the disease evolves. The goal at every stage is the same: preserve as much lung function as possible and keep you doing the things that matter to you.