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Pulmonary fibrosis

What are the causes of pulmonary fibrosis?

In most types of pulmonary fibrosis a specific cause cannot be found. One of the more common types is idiopathic pulmonary fibrosis (IPF). The word ‘idiopathic’ means there is no known cause.

In a few cases, it’s possible to identify a specific cause, such as:

Some types of pulmonary fibrosis are more likely to occur when you have another condition such as rheumatoid arthritis or scleroderma. These are sometimes called connective tissue disease related ILDs or CT-ILD

Is it inheritable?

In a very few cases, two or more members of a family may develop pulmonary fibrosis. There are many complex ways in which it’s possible to inherit a tendency to pulmonary fibrosis, and not all are well understood.

Current UK data sets suggest that less than 10% of people with pulmonary fibrosis have an inheritable disease. Genetic testing isn’t routinely available for people with pulmonary fibrosis. But there are some rare but well-known inheritable conditions that cause pulmonary fibrosis, such as dyskeratosis congenita. If, you or a family member, has one then you may be offered genetic screening.

Smoking can cause some very specific kinds of pulmonary fibrosis such as desquamative interstitial pneumonia and respiratory bronchiolitis-interstitial lung disease.

Next: what are the symptoms of pulmonary fibrosis? >

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Last medically reviewed: August 2019. Due for review: August 2022

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.