Cryptogenic fibrosing alveolitis (CFA) (or idiopathic pulmonary fibrosis) is a disease of unknown aetiology which is characterised initially by a cellular alveolar infiltrate and later by fibrosis of the alveolar walls.
The mononuclear cell infiltration of the alveoli correlates with acute symptoms whereas the fibrosis correlates with the gradual decline in lung function.
Lung function deteriorates progressively, usually over a period of years; a rapidly progressive form of the disease leads to death within months and is termed the Hamman-Rich syndrome.
It is thought that in CFA there is an abnormal immune response to an unknown stimulus.
Awareness of clinical features of idiopathic pulmonary fibrosis (1)
It usually presents with progressive dyspnoea, reduced lung volumes, bilateral lower lobe reticular opacities, and a usual interstial pneumonia pattern on histology. There is no definitive treatment and median survival is approximately 3 years (2)
Last edited 07/2019 and last reviewed 11/2019