Cryptogenic fibrosing alveolitis (CFA)

In cryptogenic fibrosing alveolitis (CFA) there is predominantly basal disease.

The spirometry is restrictive.

Lung biopsy is rarely performed, only if the clinical or radiological features are atypical.

Steroids have an effect in less than 50% of patients. Lung transplantation may be the only possible treatment for patients with progressive resistant disease.
Fig 1: "Ground glass" appearance in mid and lower zones of both lungs.  There  are also multiple "reticule-nodular" shadowings. 

Fig 2: Spirometry
Source: Fig 2

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