How Is Pulmonary Fibrosis Diagnosed?

Your healthcare provider will perform a physical exam and listen to your lungs.

• If your healthcare provider hears a crackling sound when listening to your lungs, that is a sign that you might have PF.
• Your healthcare provider will examine your joints, muscles, and skin to look for inflammation, weakness, rashes, and clubbing.
• It is also important to gather detailed information about your health, as your provider will likely ask questions about your family history of lung disease, hazardous materials you may have been exposed to in your lifetime, and diseases you’ve been treated for in the past.

Pulmonary Function Tests (PFTs) show how well your lungs are working through a series of noninvasive tests that examine your lung volume, capacity, rates of flow and gas exchange. 

• Spirometry is a test that measures the amount of air you can inhale and exhale, and how quickly and easily you can do it. You will breathe through a tube connected to a machine that will capture your data and help inform your healthcare provider how your lungs are functioning.
• Lung volume testing measures how much air your lungs can hold. To complete this test, you will sit in a clear cubicle-like box and perform a variety of breathing tests while wearing a nose clip.
• Pulse oximetry measures how much oxygen is in your blood. You will place a small device called a pulse oximeter on your fingertip to complete this test.

• Lung diffusion testing shows how well gases move between the lungs and blood. This test is completed by inhaling a small amount of carbon monoxide and a tracer gas. You will hold your breath and then rapidly exhale into a spirometer.
• Exercise testing involves activity on a treadmill or stationary bike while your heart and lung function are monitored.
• Arterial blood gas testing measures your levels of oxygen and carbon dioxide via a simple blood draw.

Tests like chest X-rays and HRCT scans can help your healthcare provider look at your lungs to see if there is any scarring and inflammation.

• Chest X-rays can sometimes show lung fibrosis, however they may not detect early or mild PF. Additionally, they cannot provide details on the pattern of PF important for identifying potential causes. 
• A chest CT (computed tomography) is a specialized imaging test that provides sharper and more detailed pictures than a standard chest X-ray. An HRCT, or high-resolution CT is a specialized CT scan that provides additional fine details of the lungs and is an important component of diagnosing PF and the type of PF you have.

Your healthcare provider may also perform an echocardiogram (ECHO), which uses sound waves to look at your heart function. This test detects pulmonary hypertension, a condition that can accompany PF, or abnormal heart function. 

Sometimes the above tests are not enough to find the cause of your symptoms, or your healthcare provider may need to confirm your diagnosis through obtaining a sample of cells, tissue or fluid from your lungs.

• Bronchoscopy is a procedure which uses a thin tube inserted down your throat or nose to collect a sample of cells or fluid from your lung. A healthcare provider may perform a bronchoalveolar lavage (BAL) to collect fluid and cell samples from the lungs. During a BAL, the healthcare provider injects salt water through a bronchoscope into a section of your lung and then suctions it out to be analyzed. 
• Lung biopsy is more invasive, but sometimes necessary to make a proper diagnosis. This may be completed by video assisted thoracoscopic surgery (VATS) or a thoracotomy, which is more invasive.

Your healthcare provider may want to run additional tests to see if you have any other health conditions.

• An esophagram (an X-ray of the esophagus) is done to see if you have a disorder that affects the esophagus, like gastroesophageal reflux disease (GERD), which can accompany certain forms of PF.
• You might be given a skin or a blood test for tuberculosis (TB), which can have some of the same symptoms as PF.
• Blood tests help determine if your liver and kidney function are normal, and to test for other conditions, including autoimmune disorders.