G: What are the treatments for PF?

Treatments for pulmonary fibrosis include both non-drug approaches – such as pulmonary rehabilitation and tailored oxygen therapy – to help manage symptoms, as well as medications like Nintedanib and Pirfenidone that can slow the progression of lung scarring. For patients whose fibrosis is linked to autoimmune disease, treating the underlying condition is essential, and in rare, advanced cases, lung transplantation may be considered.

 

🎥 Watch the Video: What are the treatments for pulmonary fibrosis?

 

Professor Porter discusses the following:

• What are the treatments for pulmonary fibrosis?
• Are there non-drug treatments for pulmonary fibrosis?
• Can pulmonary rehab help in pulmonary fibrosis?
• How can exercising help pulmonary fibrosis patients?
• Does oxygen therapy work in pulmonary fibrosis?
• What is Nintedanib / Ofev?
• What is Pirfenidone / Esbriet?
• How do Nintedanib and Pirfenidone work in pulmonary fibrosis?
• What are the side effects of Nintedanib and Pirfenidone?
• Can you manage the side effects of Nintedanib and Pirfenidone?
• Can steroids help in pulmonary fibrosis?
• Are the treatments different for pulmonary fibrosis if you have an underlying cause?
• Is lung transplantation a cure for pulmonary fibrosis?
• Which pulmonary fibrosis patients are eligible for a lung transplant?

 

Full Video Transcript

When we think about treatment, I always think about non-drug treatments and drug (or medication) treatment. In the non-drug treatment, we include pulmonary rehabilitation. So, these are exercise classes which encourage you to do more exercise so that your muscles become more efficient and they need less oxygen.  Also, within the non-medication group, we include oxygen which is itself really a medication. That can be prescribed either at rest if you have very severe lung damage and you need oxygen all the time, or just overnight or when you exercise. These will all help you with the symptoms, but they won’t actually reverse the fibrosis.

For drugs or medications for fibrosis, we have two drugs that won’t reduce the fibrosis but will slow it down so it doesn’t get worse so quickly. And these drugs are Nintedanib (or Ofev) and Pirfenidone (or Esbriet). They work in slightly different ways, but they have remarkably similar impact. They reduce the way in which the lungs fibrous by about 50%, and both of them do have side effects, unfortunately.

Pirfenidone (or Esbriet) makes your skin very sensitive to sunlight – so we advise that you wear a hat out in the summer and you use factor 50 sunscreen pretty much all year round if you expose yourself to the sun. Nintedanib (or Ofev) has gastrointestinal side effects so it can cause nausea, diarrhoea and weight loss.

However, we have a lot of experience now with managing the side effects of these drugs so we can help you by reducing the dose or taking drug holidays or using other medication to help the side effects, or modifying your diet appropriately. So, in most patients we’ll say – start with a low dose of the drug and see how you get on before you decide not to take it because you’re worried about the side effects, as some people don’t have a problem at all.

The other drugs we use are in the patients who have inflammatory disease that’s causing lung fibrosis. So, those patients with an underlying autoimmune rheumatic disease, and the drugs that we use there will be the drugs that we use to treat their underlying disease. For example, with rheumatoid arthritis, these medications will include Prednisolone or steroids, and also drugs such as Rituximab or anti B cell therapies, which are becoming standard use in rheumatoid arthritis, and also appear to reverse some of the lung scarring that we see in these diseases.

In very, very selected cases. We can refer patients with severe end stage pulmonary fibrosis for lung transplantation. But this is really only suitable for a handful of people who are at the younger end of the spectrum – patients in their 50s and early 60s. And what I always say to these patients is a lung transplant is not like changing your batteries, you won’t suddenly feel completely new. You’re exchanging one chronic lung disease for another. So, you will remain on medication all your life and on hospital follow up all your life. But in most cases, the advantages of lung transplant are far greater than the disadvantages in those patients we put forward for that very specific treatment.

 

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[Video published December 2025]