IPF Study Team

About Us

We are investigating the genetics of pulmonary fibrosis and discovering ways to intervene at the earliest stages of disease before scarring becomes irreversible.

What is idiopathic pulmonary fibrosis? Idiopathic pulmonary fibrosis (IPF) is a condition that causes progressive scarring of the lungs. As a result, fibrous scar tissue builds up in the lungs, affecting the lung's ability to provide the body with necessary amounts of oxygen.

What are symptoms of pulmonary fibrosis? Symptoms and signs of IPF often occur gradually and include:

• Shortness of breath, particularly during or after physical activity
• Chronic dry hacking cough
• Crackles in the lungs heard through a stethoscope
• Rounding of the fingernails, a condition called “clubbing”

How can I find out if I have pulmonary fibrosis? The diagnosis of IPF requires input from many members of the medical team including pulmonologists, rheumatologists, radiologists, and in many cases, pathologists experienced in the evaluation of patients with interstitial lung disease. In making a diagnosis of IPF, your doctor will start by conducting a thorough history and physical examination. This will include discussing other medical problems you have that could be related to lung scarring (e.g. connective tissue diseases), reviewing medications and potential occupational and environmental exposures, listening to your chest with a stethoscope to check for extra sounds (crackles), and carefully examining your skin and joints. In addition to a careful medical history and physical examination, the following tests may be conducted by your physician to help make an accurate diagnosis.

• Pulmonary Function Testing (PFT)
• High Resolution Computed Tomography (HRCT) scan
• Serological Tests
• Six Minute Walk Testing
• Bronchoscopy
• Surgical Lung Biopsy

What is a CT scan? A CT scan is an imaging tool that allows us to create a picture of certain parts of your body.

Are all types of pulmonary fibrosis the same? No, there are many different types of pulmonary fibrosis and idiopathic pulmonary fibrosis is a specific type of pulmonary fibrosis. In order to figure out which type of pulmonary fibrosis you have, a comprehensive medical history, physical examination and tests (as described above) are required.

What is the role of genetics in pulmonary fibrosis? With our research efforts, we are beginning to understand the role of genetics in the development of pulmonary fibrosis. There are some genetic tests that are clinically available, we recommend discussing with your doctor whether or not these are important in your care.