What are the causes of pulmonary fibrosis?

On this page we explain the different possible causes for pulmonary fibrosis (the scarring of the lungs).

In most types of pulmonary fibrosis, a specific cause cannot be found. 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.

There are lots of different types of pulmonary fibrosis, with different causes. But often there is no known cause.

Is pulmonary fibrosis hereditary?

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 form of the 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.

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