Pulmonary Fibrosis can be difficult to diagnose as the symptoms are similar to other lung diseases, so it is important to see a specialist to get an expert opinion. 

If you need help finding a specialist visit the Pulmonary Fibrosis Care Center Network.

There are three consequences of PF. Doctors use these consequences to recognize that someone has PF.

Stiff Lungs

Scar tissue and inflammation make your lungs stiff. Stiff lungs are hard to stretch, so your breathing muscles have to work extra hard just to pull air in with each breath. Your brain senses this extra work, and it lets you know there’s a problem by triggering a feeling of breathlessness(or “shortness” of breath) while exerting yourself. Also, stiff lungs hold less air (they shrink up a bit). Doctors take advantage of this “shrinking” to diagnose and track the disease using breathing tests (called Pulmonary Function Tests) that measure how much air your lungs can hold. The more scar tissue your lungs have, the less air they will hold.

Low oxygen levels in blood

Scar tissue blocks the movement of oxygen from the inside of your air sacs into your bloodstream. For many people living with pulmonary fibrosis, oxygen levels are only reduced a little bit while resting, but their oxygen levels drop quite a bit during exertion. The brain can sense these low oxygen levels, triggering breathlessness. Doctors will check your oxygen levels to see if they drop after walking, a clue that PF might be present. Doctors also often prescribe oxygen to be used through a nasal cannula or a facemask during exertion and sleep for those with PF. As pulmonary fibrosis progresses, oxygen may be needed 24 hours a day.

"Crackles"

Your doctor may have told you that “crackles” were heard in your lungs. Crackles (also called “rales”) sound like Velcro being pulled apart. They are heard in many lung diseases because any type of problem affecting the air sacs (such as PF, pneumonia, or a buildup of fluid in the lungs from heart failure) can cause crackles. Some people with pulmonary fibrosis don’t have crackles, but most do.

Once your doctor recognizes that you might have pulmonary fibrosis, the next step is to try to diagnose the specific kind of PF you have— there are more than 200 different kinds. Doctors typically start by asking many questions, performing a careful physical examination, and ordering a lot of blood tests.

A doctor will also use a special kind of X-ray of the chest, called a high-resolution computed tomography (HRCT) scan, so that they can see what your lung tissue looks like. HRCT scans give a close-up view of the lungs, providing more detail than routine CT scans (also known as CAT scans). Healthy lung tissue looks nearly black on a CT scan. Scar tissue and inflammation both appear grey or white. Many forms of PF look similar on a CT scan to the untrained eye, but subtle findings on HRCT scans are critically important when trying to identify which type of PF you have.

Sometimes, even after all of the testing is complete, a doctor will still not have an answer and will have to perform a lung biopsy to sort out which of the 200 different types of pulmonary fibrosis you have. When indicated, a lung biopsy can also help you and your doctor decide which treatments might be helpful.

For information about Treatment Options visit the Pulmonary Fibrosis Foundation website here.