Idiopathic pulmonary fibrosis (IPF) is a lung disease that results from the formation of scar tissue deep inside the lungs.
The scarring gets progressively worse. This makes it more difficult to breathe and keep adequate levels of oxygen in the bloodstream.
Ongoing low oxygen levels cause a variety of complications throughout the body. The main symptom is shortness of breath, which can lead to fatigue and other problems.
IPF is a progressive disease, which means symptoms worsen over time, and early treatment is key. There’s currently no cure for IPF, and scarring can’t be reversed or removed.
However, treatments are available that help to:
- support a healthy lifestyle
- manage symptoms
- slow disease progression
- maintain quality of life
Medical treatment options include two approved antifibrotic (anti-scarring) drugs.
Pirfenidone is an antifibrotic drug that can slow the progression of lung tissue damage. It has antifibrotic, anti-inflammatory, and antioxidant properties.
Pirfenidone has been linked to:
Nintedanib is another antifibrotic drug similar to pirfenidone that has been shown in clinical trials to slow the progression of IPF.
For most people with IPF who don’t have underlying liver disease, pirfenidone or nintedanib are the approved treatments.
Current data is insufficient to pick between pirfenidone and nintedanib.
When choosing between the two, your preference and tolerances should be considered, particularly regarding potential negative effects.
These include diarrhea and liver function test abnormalities with nintedanib and nausea and rash with pirfenidone.
Corticosteroids, like prednisone, can reduce inflammation in the lungs but are no longer a common part of routine maintenance for people with IPF as they haven’t been proven to be effective or safe.
N-Acetylcysteine (oral or aerosolized)
N-Acetylcysteine is an antioxidant that has been studied for usage in people diagnosed with IPF. Results from clinical trials have been mixed.
Similar to corticosteroids, N-Acetylcysteine is no longer commonly used as part of routine maintenance.
Other potential drug treatments include:
- proton pump inhibitors, which block the stomach from producing acid (inhalation of excess stomach acid is linked and may contribute to IPF)
- immune suppressants, such as mycophenolate and azathioprine, which can treat autoimmune disorders and help prevent the rejection of a transplanted lung
Your doctor may also recommend other treatment options. Oxygen treatment might help you breathe easier, especially during exercise and other activities.
Additional oxygen can reduce problems related to low levels of oxygen in the blood such as fatigue in the short term.
Other benefits are still being studied.
You may be a candidate for a lung transplant. Lung transplants were once reserved for younger recipients. But now they’re commonly offered to people over age 65 who are otherwise healthy.
There are several new potential treatments for IPF under investigation.
You have the option of applying to a variety of clinical trials that are looking to find new ways to prevent, diagnose, and treat a wide range of lung diseases, including IPF.
You can find clinical trials at CenterWatch, which tracks major research on searchable topics.
Lifestyle changes and other nonmedical treatments can help you stay healthier and improve your quality of your life.
Here are some recommendations.
Lose weight or maintain a healthy weight
Talk to your doctor about healthy ways to reduce or manage your weight. Being overweight can sometimes contribute to breathing difficulties.
Smoking is one of the worst things you can do to your lungs. Now, more than ever, it’s critical to stop this habit from causing more damage.
Get annual vaccinations
Talk to your doctor about yearly flu and updated pneumonia and whooping cough (pertussis) vaccines. These can help protect your lungs from infection and further damage.
Monitor your oxygen levels
Use an at-home pulse oximeter to monitor your oxygen saturation. Often the goal is to have oxygen levels at or above 90 percent.
Participate in pulmonary rehabilitation
Pulmonary rehabilitation is a multifaceted program that has become a staple of IPF treatment. It aims to improve everyday life for people with IPF as well as to reduce shortness of breath both at rest and with exercise.
Key features include:
- breathing and conditioning exercises
- stress and anxiety management
- emotional support
- nutritional counseling
- patient education
There are also support systems. These can make a big difference in your quality of life and outlook regarding living with IPF.
The Pulmonary Fibrosis Foundation has a searchable database of local support groups along with several online communities.
These resources are invaluable as you come to terms with your diagnosis and the changes it can bring to your life.
While there’s no cure for IPF, there are treatment options to manage your symptoms and improve your quality of life. These include:
- medical interventions
- lifestyle changes