Table 7.

Cause of death in 15 CVID patients with interstitial lung disease

Cause of death	CVID phenotype
Marked myocardial hypertrophy and sudden death	IO
Terminal bronchopneumonia	IO
Chest infection and NHL after benign nodular lymphoid hyperplasia 9 years previously	MALIG
DLCL after MALToma 11 years previously	MALIG
NHL ILD diagnosis 15 years previously	MALIG
NHL ILD diagnosis 12 years previously	MALIG
NHL ILD diagnosis 5 years previously	MALIG
End stage fibrotic lung	LP and CYTO
Streptococcus pneumoniae pericarditis and lung fibrosis	LP
Septicaemia and lung fibrosis	LP and CYTO
Widespread lymphocytic infiltration; CT showed LIP still	LP
Cirrhosis and pulmonary fibrosis	LP
Liver failure and ILD	LP
Respiratory failure	LP
Gut perforation after steroids given subsequent to lung bx	LP

IO = infections only CVID phenotype; MALIG = lymphoid malignancy; LP = lymphoproliferative CVID phenotype; CTY = cytopenia CVID phenotype. Pretreatment with corticosteroids; included in final histological patterns