There are many different types of Pulmonary Fibrosis that need to be distinguished from Idiopathic Pulmonary Fibrosis.

Pulmonary fibrosis can occur in association with autoimmune or connective tissue diseases such as rheumatoid arthritis, scleroderma (systemic Sclerosis), and Sjogren’s syndrome. It can also result from certain occupational exposures, including asbestos (which is known as asbestosis) as well as coal dust or silica dust (pneumoconiosis).

In addition, some medications may cause lung fibrosis, such as amiodarone, nitrofurantoin and bleomycin.

Pulmonary fibrosis may also occur following inhalation of organic dusts (e.g. from moulds or birds) which causes hypersensitivity pneumonitis (extrinsic allergic alveolitis). However in many people the cause of their pulmonary fibrosis cannot be identified and then it is known as idiopathic pulmonary fibrosis (IPF).

Why does pulmonary fibrosis occur?

There is much research focused on trying to improve our understanding of the processes and mechanisms involved in the development of pulmonary fibrosis. From this work, we believe that pulmonary fibrosis occurs as a consequence of damage to the cells that line the air sacs or alveoli in these lungs, which are called alveolar epithelial cells.