Diagnosing Pulmonary Fibrosis


2010 Update – An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

Because patients with Pulmonary Fibrosis experience symptoms and also have an underlying pattern of scarring similar to those of other lung disorders, Pulmonary Fibrosis can be difficult to diagnose. In fact, it was not until 2000 that Pulmonary Fibrosis was classified as a distinct clinical disorder by a panel of experts sponsored by the American Thoracic Society (ATS) and European Respiratory Society (ERS). Until recently, the medical community has had no agreed-upon standards for the diagnosis of Pulmonary Fibrosis. Consequently, other related lung diseases were often incorrectly classified as Pulmonary Fibrosis. With new diagnostic standards now in place, the recognition and management of Pulmonary Fibrosis should be substantially improved.

To diagnose Pulmonary Fibrosis, your doctor will take a complete medical history and perform a thorough physical examination. During this exam, the doctor will use a stethoscope to listen to your chest to try to determine if your lungs produce any abnormal sounds when you breathe. He or she may then order one or more of the following diagnostic tests or procedures:

Pulmonary Fibrosis can be difficult to diagnose.  An estimated 50% of cases are initially misdiagnosed as another form of respiratory disease.


Chest imaging Use of radiologic machines to take pictures of your lungs, such as x-ray or High Resolution Computer Tomography (HRCT) To view lung structures look for scar tissue and assess patterns of scarring
Pulmonary function test A test using a device with a mouthpiece to measure a patient’s breathing capacity To measure the degree of impairment in lung function
Arterial blood gas test A measurement of oxygen and carbon dioxide levels in blood taken from an artery in the wrist To determine how well the lungs are performing vital gas exchange
Exercise Test
(or desaturation study)
A test in which the patient is monitored while using a treadmill or stationary bicycle To measure how well the lungs and heart respond to physical activity and evaluate oxygen levels with exertion
Six Minute Walk Test (SMWT) A test where a patient walks on a flat surface as far as possible in six minutes To measure the distance you are able to walk as well as lung function during the walk.
Bronchoalveolar lavage (BAL) A “lung-washing” procedure conducted through a flexible tube (bronchoscope) inserted into the airways through the nose or mouth; fluid (salt water) is injected into the lungs and then removed for inspection To examine cells and fluid to look for signs of inflammation in the lungs, or markers of disease activity
Lung biopsy A procedure in which a tissue sample is obtained through a bronchoscope (see BAL, above) or by means of a small surgical incision (VATS- video-assisted thoracic surgery) between the ribs (open-lung biopsy) To obtain a sample of lung tissue for direct examination

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