Cryptogenic fibrosing alveolitis
Cryptogenic fibrosing alveolitis (CFA) is a fibrosing lung disease and 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 have been excluded.
On imaging consider other types of fibrosing alveolitides such as
- fibrosing alveolitis associated with systemic sclerosis (FASSc) - may have lesser upper zone involvement 2
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- 2. Chan TY, Hansell DM, Rubens MB et-al. Cryptogenic fibrosing alveolitis and the fibrosing alveolitis of systemic sclerosis: morphological differences on computed tomographic scans. Thorax. 1997;52 (3): 265-70. Thorax (link) - Free text at pubmed - Pubmed citation
- 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