What is idiopathic pulmonary fibrosis and what causes it?
Idiopathic pulmonary fibrosis used to be called cryptogenic fibrosing alveolitis. The exact cause is not known (hence the term idiopathic). It was thought that inflammation within the alveoli played a big role in the development of idiopathic pulmonary fibrosis and that this inflammation led to scarring and fibrosis. This led to the name cryptogenic fibrosing alveolitis. However, treatments that help reduce inflammation are not always effective. Therefore, the role of inflammation has recently been brought into question. The current thinking is that somehow the cells that line the alveoli are damaged in some way. The cells then try to ‘heal’ themselves. But, this healing process becomes out of control, causing thickening and damage to the walls of the alveoli, and fibrosis (scarring) of the alveoli and lung tissue. The thickening and scarring reduces the amount of oxygen that can pass into the blood vessels from affected alveoli. Therefore, as the disease progresses, less oxygen than n
It is useful to understand what the following words mean: • Idiopathic and cryptogenic mean ‘of unknown cause’. • Pulmonary means ‘affecting the lungs’. • Fibrosis means ‘thickening’ or ‘scarring’. • Alveolitis means ‘inflammation of the alveoli’. Idiopathic pulmonary fibrosis used to be called cryptogenic fibrosing alveolitis. The exact cause is not known (hence the term idiopathic). It was thought that inflammation within the alveoli played a big role in the development of idiopathic pulmonary fibrosis and that this inflammation led to scarring and fibrosis. This led to the name cryptogenic fibrosing alveolitis. However, treatments that help to reduce inflammation are not always effective. Therefore, the role of inflammation has recently been brought into question. The current thinking is that somehow the cells that line the alveoli are damaged in some way. The cells then try to heal themselves. But, this healing process becomes out of control, causing thickening and damage to the wa
