Along with a persistent cough, shortness of breath is among the most common symptoms associated with Pulmonary Fibrosis (PF).1 Referred to medically as dyspnea,2 shortness of breath is symptomatic of a wide range of diseases and conditions, varying from serious pulmonary diseases to simply being out of shape. However, dyspnea as a symptom of pulmonary fibrosis is generally progressive in that the severity of feeling “breathless” increases over time.1

PF patients often notice breathlessness when exercising or exerting themselves physically, but as the disease progresses, dyspnea begins to occur even when engaging in simple tasks such as walking, bathing, or speaking. In later-stage shortness of breath in pulmonary fibrosis, patients may even experience the symptom while at rest.4

While the severity of shortness of breath can increase over the course of the disease, severe bouts of dyspnea may also occur in the form of acute exacerbations as well.3 Exacerbations can last days or even weeks, requiring urgent care.

Why Does Shortness of Breath Occur in Pulmonary Fibrosis?

Pulmonary fibrosis is characterized by the build-up of scar tissue in the air sacs (alveoli) of the lungs. The scarring, which can be caused by several factors or be of unknown origin (known as “idiopathic” pulmonary fibrosis) inhibits the lungs from effectively distributing oxygen from the air you breathe into the bloodstream.3 As a result, PF patients experience breathlessness due to the lower levels of oxygen in the blood — an involuntary response caused by the brain when carbon dioxide levels rise and oxygen levels decrease.

How is Shortness of Breath Treated?

Dyspnea is often controlled by prescribing medications such as corticosteroids (steroids), or, in the event of a lung infection, antibiotics. In addition to drugs, oxygen therapy (when a supply of oxygen is provided to the patient via a small air tube affixed to the nostrils), and pulmonary rehabilitation including lung exercises are used.5

In more advanced cases or when treating serious exacerbations, a mechanical ventilator may be used to carefully regulate the levels of oxygen and carbon dioxide present in the lungs. Lung transplants are also an option when the scar tissue in the lungs progresses to the point where oxygen cannot be adequately supplied to the blood via breathing, even with the aid of medications, oxygen, or ventilation.

The two FDA-approved therapies for Idiopathic Pulmonary Fibrosis, Esbriet and Ofev, do not address the symptom of dyspnea directly. However, as the only two disease modifying therapies for IPF, both drugs slow down the rate of progression of fibrosis in the lungs, which in turn addresses the number of dyspnea exacerbations that IPF patients will experience over time.6

Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. 

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  1. http://www.pulmonaryfibrosis.org/life-with-pf/about-pf
  2. http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/shortness-of-breath/
  3. http://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/symptoms-causes/dxc-20211754
  4. https://www.nhlbi.nih.gov/health/health-topics/topics/ipf/signs
  5. https://www.nhlbi.nih.gov/health/health-topics/topics/ipf/treatment
  6. http://wgntv.com/2014/11/05/two-new-drug-options-give-hope-to-those-suffering-pulmonary-fibrosis/