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Pulmonary Fibrosis




Both my mom and her sister had Pulmonary Fibrosis. They both smoked heavily most of their lives. I never smoked, but was subjected to second hand smoke until 15 years ago. What are the chances of me developing this disease?


There are many interstitial lung diseases that can cause fibrosis. One of the most common is idiopathic pulmonary fibrosis. About 10-15% of cases of idiopathic pulmonary fibrosis are inherited. If both your mother and aunt had idiopathic pulmonary fibrosis, then there is a good chance that it is inherited within the family.

However, just because there is idiopathic pulmonary fibrosis in the family does not mean that you (or any of your brothers and sisters) will develop it. It appears that there are several genes than can predispose a person to developing idiopathic pulmonary fibrosis and at present, only one of them is available for commercial genetic testing (presently available from Ambry Genetics).  This gene only accounts for about 1/10 cases of inherited pulmonary fibrosis.

In your case, seeing a physician regularly to listen to the lung sounds for abnormal "crackles" is the best way to screen for pulmonary fibrosis. Should you have persistent cough or shortness of breath, a high resolution chest CT scan and/or pulmonary function tests can tell if you have the disease. By avoiding cigarette smoke and dusty air at work and at home, you may be able to reduce your chances of developing pulmonary fibrosis.

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Response by:

James N Allen, Jr, MD James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University