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Symptoms and Diagnosis


Most patients with pulmonary fibrosis present with shortness of breath. Other symptoms that are often present at the time of diagnosis include cough and fatigue.


Physical Examination

Some physical examination findings that may support a diagnosis of pulmonary fibrosis include crackles in the lungs and changes in the nail beds referred to as "clubbing."

CT Imaging

Pulmonary fibrosis is typically diagnosed using CT imaging but may also be diagnosed on examination of lung tissue after a lung biopsy. Given the improvement in CT imaging, a lung biopsy may not be required to make a diagnosis of pulmonary fibrosis. Pulmonologists and radiologists look for characteristic patterns of abnormalities in the lung tissue that may identify a specific underlying cause of the pulmonary fibrosis. A typical pulmonary fibrosis pattern of abnormality seen in the lung tissue on a CT scan includes increased densities in the periphery of the lung that are referred to as "reticular changes."


Bronchoscopy is an outpatient procedure that involves passing a fiber-optic camera into the trachea and lower airways. Bronchoscopy may be used to inspect the airways for abnormalities or to obtain samples of lung fluid or tissue. Bronchoscopy may be used to determine the etiology of your lung disease. It may also be used to diagnose respiratory infections, particularly in patients who are taking immuno-suppressive drugs. However, it is not necessarily useful for diagnostic evaluation of all ILDs and/or all forms of pulmonary fibrosis. Your pulmonologist should discuss with you whether a bronchoscopy would be of use to diagnose your lung disease.

Lung Biopsy

A lung biopsy may be performed to help determine the cause of your lung disease. The decision whether to pursue a lung biopsy is often difficult as there are many factors that will determine the likelihood of defining a cause of the lung disease and the risk of suffering an adverse event during or after the surgery. Your pulmonologist should discuss the benefits and risks of the procedure with you so that you can make an informed decision about whether to undergo a lung biopsy. If a lung biopsy is performed, a lung pathologist would review the biopsy specimen to determine what pattern(s) of lung disease or lung injury exist in the sample. For example, usual interstitial pneumonia (UIP) is a pattern of lung disease that is seen in many patients with pulmonary fibrosis. Your pulmonologist would then attempt to correlate the pattern of lung disease from the biopsy with a specific etiology (if one is readily apparent). In the case of UIP, if there is no definable source for the lung disease, it would be determined to be idiopathic and therefore most likely represent idiopathic pulmonary fibrosis.