Shortness of breath can be an early symptom of idiopathic pulmonary fibrosis (IPF), a rare and serious lung disease that generally affects middle-aged to older adults between the ages of 50 and 70. Shortness of breath can also be a symptom of other chronic conditions like:

  • heart disease
  • asthma
  • chronic obstructive pulmonary disease (COPD)

With IPF, the small air sacs in your lungs called alveoli become thick and stiff or scarred. This also means that your lungs have difficulty moving oxygen into the bloodstream and to your organs. The scarring in the lungs often gets worse as time goes on. Breathing and oxygen delivery, as a result, become more impaired.

There’s currently no cure for IPF. The course of the disease varies greatly among individuals. Some people experience:

  • rapid worsening
  • episodes of on and off worsening
  • slow progression
  • disease stability for years

While experts agree that the median length of survival in individuals with IPF is typically around 3 to 5 years from diagnosis, people in disease stability can live much longer. Respiratory failure is the most common cause of death in IPF, but other causes can include:

One of the earliest symptoms of IPF is shortness of breath. You may notice you are winded while walking down the street or going upstairs. You may have difficulty breathing while performing other physical tasks and need to take breaks to complete them. This happens because IPF causes stiffening or thickening and scarring within your lungs. As your lungs become more rigid, it’s harder for them to inflate and they can’t hold as much air.

Shortness of breath is also called dyspnea. In the later stages of the disease, shortness of breath may make it difficult to talk on the phone, eat, or even take full breaths while at rest.

Coughing is another early symptom of IPF. This cough is typically dry and does not bring up any phlegm or mucus.

Other symptoms of the disease, can include:

  • abnormal breathing sounds (crackles)
  • clubbing of fingers or toes
  • fatigue
  • muscle and joint aches
  • unexplained weight loss

Experts agree that the course of IPF can be unpredictable.

If you’re experiencing shortness of breath or have any of the other symptoms of IPF, make an appointment with your doctor for a physical exam. They may refer you to a pulmonologist — a lung specialist who can evaluate:

  • X-rays
  • breathing tests
  • heart tests
  • biopsies
  • blood oxygen level tests

You may want to answer the following questions before your appointment so you can give your doctor a better picture of your medical history:

  • What are your symptoms? When did they start?
  • What is your current or previous occupation?
  • Do you have other medical conditions?
  • What medications or supplements are you currently taking?
  • Do you smoke? If yes, how often and for how many years?
  • Do you know of family members who have had chronic lung disease or IPF specifically?
  • Is there anything else that you think your doctor should know about your health?

Shortness of breath may be an early symptom of IPF. If you’re experiencing this symptom, schedule a physical exam and evaluation with your doctor. Getting an accurate diagnosis as soon as possible may help you get treatments that can slow the progress of the disease and improve your quality of life.

If you’re diagnosed with IPF, there are a few things you can try that might help you manage the disease:

  • If you smoke, try to quit. Smoking is harmful to your lungs. Your doctor can give you support to help you stop smoking, or you can start your quitting plan today on the Centers for Disease Control and Prevention website.
  • Eat a nutritious diet. When breathing is difficult, you may not feel like eating, which can cause you to lose weight. Try to add fruits, vegetables, whole grains, low fat or fat-free dairy, and lean meats to your daily routine. You can also try eating smaller and more frequent meals.
  • Exercise regularly. Though you may worry about having shortness of breath, moving your body can actually help maintain your lung function and lower stress levels.
  • Get good rest. Sleeping and taking time to rest is just as important as exercising. It can help with your energy levels and with managing stress.
  • Consider vaccinations. The pneumonia vaccine, whooping cough vaccine, COVID-19 vaccination and booster, and flu shots can safeguard you against respiratory infections that might make your IPF worse.
  • Take medications as directed by your doctor. Make sure you’re keeping up with your appointments, reporting any new or unusual symptoms, and following any other instructions your doctor has given you.

In the past, the available medications focused on treating lung inflammation. More recent medications focus on treating lung scarring. Pirfenidone (Esbriet) and nintedanib (Ofev), two medications approved in 2014 for the treatment of pulmonary fibrosis, are often now considered the standard of care. These medications have been shown to slow the progression of the disease as well as slow the worsening of lung function.

IPF is a rare lung disease that varies in seriousness across those who are diagnosed. Knowing the symptoms of IPF is critical for early diagnosis and creating a plan to slow disease progression. If you are experiencing symptoms like shortness of breath that persists, be sure to talk with your doctor about your concerns. If you are diagnosed with IPF, work with your healthcare professional to create a treatment plan that works for you. While there is currently no cure, medical treatment and self-management strategies are available to help people with IPF.