Idiopathic Pulmonary Fibrosis: Life Expectancy and Outlook

Pulmonary fibrosis is a condition of the lungs that causes scarring of the lung tissue. The most common type of pulmonary fibrosis is known as idiopathic pulmonary fibrosis. This is a chronic lung disease that causes a buildup of scar tissue between the air sacs of the lungs. Idiopathic pulmonary fibrosis causes the damaged lung tissue to become stiff and thick, making it difficult for the lungs to work properly. This results in breathing difficulties, leading to lower levels of oxygen in the blood. There is no known cause of idiopathic pulmonary fibrosis, and diagnosis depends on the exclusion of other interstitial lung diseases. Treatment of idiopathic pulmonary fibrosis involves supportive therapy, medications, and in severe cases, a lung transplant. Let us take a closer look at the life expectancy and outlook for idiopathic pulmonary fibrosis. 

What is the Life Expectancy of People with Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a chronic lung disease that causes scar tissue to buildup deep in the lungs, especially between the air sacs.(1,2,3) This causes significant damage to the lung tissues, making them stiff and thick due to which the lungs are unable to function correctly.(4) This causes difficulty in breathing, coughing, and also leads to lower levels of oxygen present in the blood.(5,6) Lower levels of oxygen in the bloodstream are likely to tire you out, making you feel exhausted and unwell. This feeling of fatigue tends to worsen if you start avoiding physical activity to prevent feeling short of breath.(7)

The life expectancy of people with idiopathic pulmonary fibrosis is around three years.(8) However, life expectancy in people with this condition can vary. If you have idiopathic pulmonary fibrosis, then your life expectancy is going to be influenced by factors such as the progression of the disease, the severity of your symptoms, and your age. You may easily increase the three-year estimation and also improve the overall quality of your life by finding out how to best manage your symptoms and disease progression.

However, it is essential to keep in mind that there is no cure for idiopathic pulmonary fibrosis. Nevertheless, active research by the National Heart, Lung, and Blood Institute in the United States is aimed at spreading awareness of idiopathic pulmonary fibrosis, raise more funds for research, and carry out clinical trials with the aim of finding a life-saving cure for idiopathic pulmonary fibrosis.(9)

It is important to remember, though, that an early detection of idiopathic pulmonary fibrosis is also very important to slow down the progression. Furthermore, your life expectancy and disease prognosis can also be impacted if you have additional medical conditions such as pulmonary arterial hypertension (PAH) or chronic obstructive pulmonary disease (COPD), both of which are risk factors when you have idiopathic pulmonary fibrosis.(10)

Chronic obstructive pulmonary disease is also a lung disease that is characterized by chronic inflammation in the lungs and airways. COPD causes frequent cough, excessive mucus production, and an increased risk of infection. Chronic inflammation associated with COPD is known to dramatically worsen the scarring of the lungs caused by idiopathic pulmonary fibrosis.(11)

Pulmonary arterial hypertension is another serious complication that can result from idiopathic pulmonary fibrosis. The condition is characterized by high blood pressure in the blood vessels that surround your heart and lungs. Such a condition is also known to significantly impact the prognosis for idiopathic pulmonary fibrosis.(12)

There are several new anti-scarring medications, such as nintedanib (brand name OFEV) and pirfenidone (brand name Esbriet), that are being hailed for slowing down the progression of idiopathic pulmonary fibrosis in many people. However, these medications do not make any difference to a person’s life expectancy.  Researchers are continuing to look for newer combinations of drugs that may improve the disease outcome even further.(13)

The life expectancy of someone with idiopathic pulmonary fibrosis also depends on which stage of the disease you are at when you are diagnosed. There are four stages of pulmonary fibrosis, including:

  • Mild
  • Moderate
  • Severe
  • Very severe

The idiopathic pulmonary fibrosis disease stage is determined by your lung capacity at the time of diagnosis and the severity of your symptoms. This is why an early diagnosis of idiopathic pulmonary fibrosis is important because, as the disease progresses, your lung capacity starts to decrease. By the time you reach the advanced or very severe stage, you might not be able to breathe without any mechanical assistance. Diagnosis at such an advanced stage of the disease is going to reduce your life expectancy further.(14)

Another factor that affects life expectancy in people with idiopathic pulmonary fibrosis is that the risk of developing complications goes up steadily as the disease progresses. As the disease advances, you are at a significantly higher risk of developing various life-threatening complications. These including:

  • Pulmonary embolism (blood clots in the lungs)
  • Stroke and heart attack
  • Respiratory failure
  • Heart failure
  • Lung cancer(15,16)
  • Severe infections in the lungs

 Outlook for Idiopathic Pulmonary Fibrosis

It is essential to realize that idiopathic pulmonary fibrosis is a chronic and progressive disease for which there is no cure. This means that you are going to have idiopathic pulmonary fibrosis for the rest of your life. Nevertheless, the outlook for people with idiopathic pulmonary fibrosis varies greatly. While some people end up becoming sick very rapidly, others may progress at a slower rate, and the condition may take several years to advance to the next stage.

A large part of managing the outlook of idiopathic pulmonary fibrosis depends on how dedicated you are to adopting healthy lifestyle practices that can not only reduce the severity of your symptoms but also dramatically improve your outlook. Some of these lifestyle changes include:

  • Exercising regularly
  • Losing weight and maintaining a healthy weight
  • Quit smoking if you currently do
  • Following your prescribed treatment plan dedicatedly – for example, being on top of all your medications, vaccines, and taking any vitamins and supplements as required
  • Using an oxygen monitor to maintain your oxygen saturation within an optimal range.
  • Consuming a well-balanced and healthy diet that includes lots of fresh vegetables and fruits, whole grains, healthy fats, and the right amount of vitamins and minerals.

Many people with idiopathic pulmonary fibrosis find it helpful to join a pulmonary rehabilitation support group. In many cases, it has been found that practicing breathing and conditioning exercises, along with techniques for managing anxiety, stress, and having someone to discuss your emotions following this diagnosis, can improve the outlook for idiopathic pulmonary fibrosis significantly.(17,18) 

Conclusion

Overall, getting support from different types of services such as social work and palliative care has been found to be important in improving the outlook for idiopathic pulmonary fibrosis. Pulmonary rehabilitation may also significantly improve your overall quality of life by helping you take control and manage your diet, breathing, and physical activity. All these factors can go a long way in improving your life expectancy as well. Remember that an early diagnosis is important and allows you to start treatment at the earliest. Early treatment can help slow down the progression of the disease, and with the right treatment plan and care, it is very much possible to have a good quality of life even with idiopathic pulmonary fibrosis.

References:

  1. Gross, T.J. and Hunninghake, G.W., 2001. Idiopathic pulmonary fibrosis. New England Journal of Medicine, 345(7), pp.517-525.
  2. King Jr, T.E., Pardo, A. and Selman, M., 2011. Idiopathic pulmonary fibrosis. The Lancet, 378(9807), pp.1949-1961.
  3. Collard, H.R., Moore, B.B., Flaherty, K.R., Brown, K.K., Kaner, R.J., King Jr, T.E., Lasky, J.A., Loyd, J.E., Noth, I., Olman, M.A. and Raghu, G., 2007. Acute exacerbations of idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 176(7), pp.636-643.
  4. Raghu, G., Weycker, D., Edelsberg, J., Bradford, W.Z. and Oster, G., 2006. Incidence and prevalence of idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 174(7), pp.810-816.
  5. Douglas, W.W., Ryu, J.H. and Schroeder, D.R., 2000. Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival. American journal of respiratory and critical care medicine, 161(4), pp.1172-1178.
  6. Strausz, J., Muller-Quernheim, J., Steppling, H. and Ferlinz, R., 1990. Oxygen radical production by alveolar inflammatory cells in idiopathic pulmonary fibrosis. Am Rev Respir Dis, 141(1), pp.124-128.
  7. Jackson, R.M., Gomez-Marin, O.W., Ramos, C.F., Sol, C.M., Cohen, M.I., Gaunaurd, I.A., Cahalin, L.P. and Cardenas, D.D., 2014. Exercise limitation in IPF patients: a randomized trial of pulmonary rehabilitation. Lung, 192(3), pp.367-376.
  8. Ley, B., Collard, H.R. and King Jr, T.E., 2011. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 183(4), pp.431-440.
  9. Nhlbi.nih.gov. 2020. Idiopathic Pulmonary Fibrosis | NHLBI, NIH. [online] Available at: <https://www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis> [Accessed 9 September 2020].
  10. Chilosi, M., Poletti, V. and Rossi, A., 2012. The pathogenesis of COPD and IPF: distinct horns of the same devil?. Respiratory research, 13(1), pp.1-9.
  11. Chilosi, M., Carloni, A., Rossi, A. and Poletti, V., 2013. Premature lung aging and cellular senescence in the pathogenesis of idiopathic pulmonary fibrosis and COPD/emphysema. Translational Research, 162(3), pp.156-173.
  12. Lettieri, C.J., Nathan, S.D., Barnett, S.D., Ahmad, S. and Shorr, A.F., 2006. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest, 129(3), pp.746-752.
  13. Sgalla, G., Iovene, B., Calvello, M., Ori, M., Varone, F. and Richeldi, L., 2018. Idiopathic pulmonary fibrosis: pathogenesis and management. Respiratory research, 19(1), p.32.
  14. Homma, S., Sugino, K. and Sakamoto, S., 2015. The usefulness of a disease severity staging classification system for IPF in Japan: 20 years of experience from empirical evidence to randomized control trial enrollment. Respiratory investigation, 53(1), pp.7-12.
  15. Tzouvelekis, A., Spagnolo, P., Bonella, F., Vancheri, C., Tzilas, V., Crestani, B., Kreuter, M. and Bouros, D., 2018. Patients with IPF and lung cancer: diagnosis and management. The Lancet Respiratory Medicine, 6(2), pp.86-88.
  16. Karampitsakos, T., Tzilas, V., Tringidou, R., Steiropoulos, P., Aidinis, V., Papiris, S.A., Bouros, D. and Tzouvelekis, A., 2017. Lung cancer in patients with idiopathic pulmonary fibrosis. Pulmonary pharmacology & therapeutics, 45, pp.1-10.
  17. Holland, A.E., Hill, C.J., Glaspole, I., Goh, N. and McDonald, C.F., 2012. Predictors of benefit following pulmonary rehabilitation for interstitial lung disease. Respiratory medicine, 106(3), pp.429-435.
  18. Nishiyama, O., Kondoh, Y., Kimura, T., Kato, K., Kataoka, K., Ogawa, T., Watanabe, F., Arizono, S., Nishimura, K. and Taniguchi, H., 2008. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology, 13(3), pp.394-399.

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