TABLE 3

Lung histopathological findings

FeaturesSubjects (n with finding/n tested)
Number of biopsies available for review10 (10/11)
Type of biopsy
 Open lung biopsy8 (8/10)
 Transbronchial biopsy2 (2/10)
Follicular bronchiolitis7 (7/10)
Alveolar haemorrhage4 (4/10)
Airspace enlargement/cystic changes2 (2/10)
Acute lung injury with capillaritis2 (2/10)#
Interstitial fibrosis2 (2/10)
Nondiagnostic1 (1/10)

#: both subjects with this finding were related (father and daughter); : both biopsies demonstrated non-usual interstitial pneumonitis.