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Pulmonary Fibrosis

Ideopathic Pulmonary Fibrosis

07/23/2007

Question:

My Mom died in March this year from this, now we have been told two of her siblings also have this diease. Do you have any information on heredity or possibliy whole familieas having this diease?

Answer:

This is a subject of intense research right now. We believe that between 5-10% of all cases of idiopathic pulmonary fibrosis (IPF) are inherited. It is almost always the UIP (usual interstitial pneumonitis) variant. The specific gene or genes have not yet been identified, but a research group at Duke University is actively looking for the responsible genes.

The treatment and prognosis for inherited IPF is the same as non-inherited IPF. For persons who have IPF in blood relatives, I sometimes recommend genetic counseling, but there is no test at this time to predict whether a son or daughter of a patient with IPF will also develop it later in life. My normal practice is to examine the fingers for "clubbing" and listen to the lungs for "crackles" yearly (this can be done by a primary care physician or a pulmonologist). If these or new pulmonary symptoms of persistent cough or shortness of breath develop, I will obtain pulmonary function tests and a high resolution chest CT.

 

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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