Cryptogenic fibrosing alveolitis (CFA) is a fibrosing lung disease that is characterised by inflammation and fibrosis of the alveoli and interstitium of the lungs, favouring the subpleural and basal regions.
There is some overlap in definition with the term idiopathic pulmonary fibrosis 1.
By definition, the diagnosis demands that all known causes of pulmonary fibrosis be excluded.
On imaging consider other types of fibrosing alveolitides such as
- fibrosing alveolitis associated with systemic sclerosis (FASSc): in systemic sclerosis patients; may have lesser upper zone involvement 2
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- 3. Wells A. Clinical usefulness of high resolution computed tomography in cryptogenic fibrosing alveolitis. Thorax. 1998;53 (12): 1080-7. Thorax (link) - Free text at pubmed - Pubmed citation
- 4. du Bois RM, Wells AU. Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis. Eur Respir J Suppl. 2002;32: 43s-55s. Pubmed citation