Idiopathic Pulmonary Fibrosis Treatments and Prognosis

New Treatments and Support for Idiopathic Pulmonary Fibrosis

doctor dispensing a medication implying a treatment is available
How is idiopathic pulmonary fibrosis treated?. Photo©Dragonimages

Treatments for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is not curable, but it is treatable. Thankfully, new medications have been approved just since 2014 which are making a difference in the quality of life and progression for people living with the disease. In contrast, medications used until very recently have been deemed to cause more harm than help for some people with IPF.

If you've been diagnosed with this disease, make sure you don't get discouraged by older information.

If you're interested more in the symptoms and causes of IPF, check out the following articles as well:

Goals of Treatment for Idiopathic Pulmonary Fibrosis

The damage that has occurred in IPF is by definition irreversible; the fibrosis that has occurred cannot be healed. Therefore, the goals of treatment are to:

  • Minimize further damage to the lungs. Since the underlying cause of IPF is damage followed by abnormal healing, treatment is directed at these mechanisms.
  • Improve breathing difficulty.
  • Maximize activity and quality of life.

Since IPF is an uncommon disease, it is helpful if patients can seek out care at a medical center which specializes in idiopathic pulmonary fibrosis/interstitial lung disease.  Options for treatment include:

Medications for Idiopathic Pulmonary Fibrosis

Tyrosine Kinase Inhibitors - In October of 2014, 2 medications became the first drugs approved by the FDA specifically for the treatment of idiopathic pulmonary fibrosis.

These medications, pirfenidone and nintedanib target enzymes called tyrosine kinase, and work by reducing fibrosis (antifibrotics.)  (Very simplistically, tyrosine kinase enzymes activate the growth factors that cause fibrosis, therefore these medications block the enzymes and hence the growth factors that would cause further fibrosis.) These medications were found to have several benefits:

  • They reduced disease progression by half during the year patients took the drug (it is now being studied over longer periods of time.)
  • They decrease the functional decline in lung function (the decrease in FVC is less) by half.
  • They result in fewer exacerbations of the disease
  • Patients using these drugs had better health-related quality of life.

These medications are generally tolerated quite well, which is very important for a progressive disease without a cure; the most common symptom being diarrhea.

N-Acetylcysteine - In the past, n-acetylcysteine was often used to treat IPF but more recent studies have not found this to be effective. When broken down it appears that people with some gene types may improve on the medication, while those with another gene type (another allele) are actually harmed by the drug.

Of interest is a recent study done using esomeprazole, a proton pump inhibitor, on lung cells in the lab and in rats. This medication which is commonly used to treat gastroesophageal reflux disease resulted in increased survival in both the lung cells and in rats. Since GERD is a common precursor to IPF, it's thought that acid from the stomach aspirated into the lungs may be part of the etiology of IPF.

 While this has yet to be tested in humans, certainly treatment of chronic GERD in individuals with IPF should be considered.

Lung Transplant Surgery

The use of a bilateral or single lung transplant as a treatment for IPF has been steadily increasing over the last 15 years and represents the largest group of people waiting for lung transplants in the United States. It carries significant risk but is the only treatment known at this time to clearly extend life expectancy. Currently, the median survival (time after which half of people have died and half are still alive) is 4.5 years with a transplant, though it’s likely that survival has improved during that time while medicine becomes more advanced.

The survival rate is higher for bilateral transplants than a single lung transplant, but it’s thought that this has more to do with factors other than the transplant (characteristics of the people who had 1 or 2 transplanted lungs.)  Unfortunately, in one study, 14 to 67% of people died while waiting for a transplant.

Supportive Treatment

Since IPF is a progressive disease, supportive treatment to ensure the best quality of life possible is extremely important. Some of these measures include:

  • Management of Concurrent Problems.  
  • Treatment of symptoms.
  • Flu shot and pneumonia shot to help prevent infections.
  • Pulmonary rehabilitation.
  • Oxygen therapy - Some people are hesitant to use oxygen due to the stigma, but it can be very helpful for some people with IPF. Certainly, it makes breathing easier and allows people with the disease to have a better quality of life, but it also reduces complications related to low oxygen in the blood and reduces pulmonary hypertension (high blood pressure in the arteries that travel between the right side of the heart and the lungs.)

Co-existing Conditions (Co-morbidities) and Complications

Several complications are common in people living with IPF.  These include:

Online Support Groups/Online Support Communities

Just as people with cancer often say, there is nothing like talking to another person who is coping with a disease like you.. Yet, since IPF is uncommon, there’s likely not a support group in your community.  If you are receiving treatment at a facility that specializes in IPF, there may be in-person support groups available through your medical center.

For those who do not have a support group such as this—which probably means most people with IPF—online support groups and communities are a great option. In addition, these are communities that you can seek out 7 days a week 24 hours a day when you really need to touch base with someone. Support groups are helpful in lending emotional support for many people and are also a way to keep up on the latest findings and treatments for the disease.  I’ve spoken with people in the lung cancer community who value their online friendships as they do close friends and family members. Medicine is changing.  Many advances are taking place because patients are asking for new treatments, and with social media, doctors are listening. Examples of support groups include:


The prognosis of idiopathic pulmonary fibrosis varies widely, with some people having rapidly progressive disease, and others who remain stable for many years.  It’s hard to predict what the course will be with any one patient. The median survival rate was 3.3 years in 2007 vs 3.8 years in 2011. Even without the newly approved medications, care appears to be improving.  Another study found that people aged 65 and over were living longer with IPF in 2011 than they were in 2001.

What is Something You Wish Everyone Knew About Idiopathic Pulmonary Fibrosis?

What would physicians who specialize in IPF hope everyone knew about the disease? According to Dr. Imre Noth, a physician specializing in interstitial lung diseases such as IPF at the University of Chicago: "I wish everyone knew that it’s not their fault and that there is plenty we can do for this disease. Patients should be evaluated early at ILD centers!" Since this is an uncommon disease with recent breakthroughs, seeking out a physician, or at least a second opinion in a large center which specializes in the disease could make a difference.  

Dan Castner, a survivor who has been living with the disease says: "I wish everyone knew about the dramatic lifestyle change that people with IPF experience. The ability to breathe freely is something that is taken for granted. I had never considered otherwise.  All of those daily acts I was able to do before my diagnosis such as simply walking up stairs, I now have to think twice about. I can no longer do any of the physical activities I once did that were a major part of my lifestyle; a part of what defined me in the past."

During cancer support months in the fall of each year, we hear about symptoms of cancer, and how to support people living with the disease. It seems it's about time to raise our voices about this condition as well.


Antoniou, K., Tomassetti, S., Tsitoura, E., and C. Vancheri. Idiopathic pulmonary fibrosis and lung cancer: a clinical and pathogenesis update. Current Opinions in Pulmonary Medicine. 2015 Sep 18. (Epub ahead of print).

de Boer, K., and J. Lee. Under-recognised comorbidities in idiopathic pulmonary fibrosis: a review. Respirology. 2015 Sep 13. (Epub ahead of print).

Esposito, D. et al. Idiopathic Pulmonary Fibrosis in US Automated Claims: Incidence, Prevalence and Algorithm Validation. American Journal of Respiratory and Critical Care Medicine. 2015 Aug 4. (Epub ahead of print)>

Ghebremariam, Y. et al. Pleiotropic effect of the proton pump inhibitor esomeprazole leading to suppression of lung inflammation and fibrosis. Journal of Translational Medicine. 2015. 13:249.

Hao, W., Marsh, C., and A. Friedman. A Mathematical Model of Idiopathic Pulmonary Fibrosis. PLos One. 2015 Sep 8. 10(9):e0135097.

Hutchinson, J., McKeever, T., Fogarty, A., Navaratnam, V., and R. Hubbard. Increasing global mortality from idiopathic pulmonary fibrois in the twenty-first century. Annals of the American Thoracic Society. 2014. 11(8):1176-85.

Idiopathic Pulmonary Fibrosis Clinical Research Network, Martinez, F., deAndrade, J., Anstrom, K., King, T., and G. Raghu. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. New England Journal of Medicine. 2014. 370(22):2093-101.

Kim, H., Perlman, D., and R. Tomic. Natural history of idiopathic pulmonary fibrosis. Respiratory Medicine. 2015. 109(6):661-70.

Kistler, K., Nalysnyk, L., Rotella, P., and D. Esser. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulmonary Medicine. 2014. 14:139.

Oldham, J. et al. TOLLIP, MUC5B and the Response to N-acetylcystein Among Individuals with Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 2015 Sep 2. (Epub ahead of print).

O’Riordan, T., Smith, V., and G. Raghu. Development of Novel Agents for Idiopathic Pulmonary Fibrosis: progress in target selection and clinical trial design. Chest. 2015 May 8. (Epub ahead of print).

Raghu, G. et al. Diagnosis of idiopathic pulmonary fibrosis with high-resolution CT in patients with little or no radiological evidence of honeycombing: secondary analysis of a randomized, controlled trial. Lancet Respiratory Medicine. 2014. 2(4):277-84.

Raghu, G. et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respiratory Medicine. 2014. 2(7):566-72.

Richeldi, L. et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New England Journal of Medicine. 2014. 370(22):2071-82.

Spagnolo, P., Maher, T., and L. Richeldi. Idiopathic pulmonary fibrosis: Recent advances on pharmacological therapy. Pharmacology & Therapeutics. 2015. 152:18-27.

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