There is no single best treatment for interstitial lung disease because ILD is not one disease. It is a group of over 200 conditions that cause scarring or inflammation in the lungs, and the right treatment depends entirely on which type you have and whether it is primarily inflammatory, fibrotic, or both. The main branches of treatment include anti-fibrotic medications for scarring diseases, immune-suppressing medications for inflammatory types, pulmonary rehabilitation, supplemental oxygen, and in advanced cases, lung transplantation.
Why the Type of ILD Determines Treatment
The two broadest categories that shape your treatment plan are fibrotic ILD and inflammatory ILD. Idiopathic pulmonary fibrosis (IPF) is the most common fibrotic type, and it behaves very differently from ILD caused by autoimmune conditions like rheumatoid arthritis or lupus. In autoimmune-related ILD, the immune system is actively attacking lung tissue, so calming that immune response can stabilize or even improve lung function. In IPF, inflammation is not the main driver. Scarring progresses on its own, and immune-suppressing drugs not only fail to help but can cause harm.
A newer clinical concept called progressive pulmonary fibrosis (PPF) now applies to patients with any fibrotic ILD (not just IPF) whose disease keeps worsening despite initial treatment. PPF is defined by at least two of three criteria within a single year: worsening breathing symptoms, declining lung function on breathing tests, and worsening scarring on imaging. If your ILD meets that threshold, your doctor may shift your treatment toward anti-fibrotic drugs regardless of the original diagnosis.
Anti-Fibrotic Drugs for Scarring Disease
For IPF and progressive fibrotic ILD, anti-fibrotic medications are the cornerstone of treatment. Two drugs, pirfenidone and nintedanib, have been available for several years. Neither reverses existing scarring, but both slow the rate at which your lungs lose function. A real-world study published in Respiratory Medicine found that over 24 months, patients on either drug had significantly less decline in lung capacity compared to untreated patients, with no meaningful difference between the two medications.
In October 2025, the FDA approved a third anti-fibrotic option called nerandomilast (brand name Jascayd) specifically for IPF. In two large clinical trials, patients taking nerandomilast had a significantly smaller decline in lung capacity compared to placebo. The drug is taken as a tablet twice daily. Common side effects include diarrhea, nausea, fatigue, decreased appetite, and weight loss.
Diarrhea is the most frequent complaint across all anti-fibrotic medications. For nintedanib, taking the pill with food and using over-the-counter anti-diarrheal medication like loperamide can keep symptoms manageable. Anti-nausea medication also helps. If side effects become severe, your dose can be reduced or the drug temporarily paused. Many patients find that side effects ease after the first few weeks.
Immune-Suppressing Drugs for Inflammatory ILD
When ILD is driven by an autoimmune or inflammatory condition, the goal is to quiet the immune system’s attack on the lungs. Corticosteroids like prednisone are often used first to bring active inflammation under control, but long-term steroid use causes serious side effects. So doctors typically add a steroid-sparing medication to allow the prednisone dose to come down over time.
Two of the most commonly used steroid-sparing drugs are azathioprine and mycophenolate. A study in CHEST Journal followed patients with myositis-related ILD on these drugs for up to five years. Both groups saw improvements in lung capacity, and both groups were able to reduce their prednisone doses. Azathioprine had a slight edge in lowering steroid doses (about 6.6 mg lower at three years), but it also came with notably more side effects: 33% of patients on azathioprine experienced adverse events compared to about 14% on mycophenolate. For many patients, mycophenolate’s better tolerability makes it the preferred choice.
Pulmonary Rehabilitation
Pulmonary rehabilitation is one of the most underused treatments for ILD, and the evidence supporting it is strong. These supervised programs combine structured exercise, breathing techniques, and education over a period of weeks to months. A meta-analysis of 11 randomized controlled trials found that patients who completed rehab walked meaningfully farther on a standardized six-minute walk test and reported better quality of life.
The benefits were largest in programs that lasted longer than eight weeks, were fully supervised, and included high-intensity interval training. Patients in programs with interval training gained an average of 77 meters on their walking distance, a substantial improvement that translates to real differences in daily life: being able to walk to the mailbox, get through a grocery store, or climb a flight of stairs without stopping. Quality of life scores improved well beyond the threshold considered clinically meaningful.
Rehab does not reverse scarring, but it trains your body to use the lung capacity you have more efficiently. It also builds the muscle strength and cardiovascular fitness that deteriorate when breathlessness makes you less active.
Supplemental Oxygen
As ILD progresses, your lungs become less efficient at transferring oxygen into your blood. You may notice this first during exercise or walking, then eventually at rest. British Thoracic Society guidelines recommend targeting oxygen saturation levels of 94 to 98% during acute flare-ups of pulmonary fibrosis, or the highest achievable level if those targets are not realistic.
A sudden drop of 3% or more in your oxygen saturation, even if you are still within a normal range, should prompt a closer evaluation because it can signal an acute worsening. Many ILD patients use portable oxygen concentrators during physical activity long before they need oxygen around the clock. This allows you to stay more active and can make pulmonary rehabilitation more effective.
Lung Transplantation
For people with advanced ILD who continue to decline despite medication and other treatments, lung transplantation remains the only option that can dramatically extend life. Referral typically happens when lung function drops below specific thresholds. A multicenter study found that nearly 99% of ILD patients referred for transplant evaluation had a lung capacity below 80% of predicted or a gas exchange measurement below 40% of predicted.
The key with transplant evaluation is timing. The process from referral to listing to receiving a donor organ can take months or longer, and patients need to be well enough to survive the surgery and recovery. If your lung function is declining steadily despite treatment, early referral gives you more options, not fewer.
How Treatment Is Monitored
Once you start treatment, your medical team will track your lung function at regular intervals using breathing tests (spirometry and gas exchange measurements) and periodic imaging. For autoimmune-related ILD, breathing tests are typically recommended every three to six months during the first year, then less frequently once your condition stabilizes. For other forms of ILD, the interval is usually every three to twelve months depending on how active or aggressive your disease appears.
Repeat CT scans are not done on a fixed schedule. They are ordered when your symptoms change, your breathing test numbers decline, or your doctor needs a clearer picture of whether scarring is progressing. Monitoring is not just about checking numbers. It is about catching progression early enough to adjust your treatment plan, whether that means adding an anti-fibrotic drug, changing your immunosuppressive regimen, or beginning the transplant conversation.