Table 2

Underlying and immediate causes for death in patients with idiopathic pulmonary fibrosis (IPF) and other fibrosing interstitial lung disease (FILD)

Total
n=107
IPF
n=70
Other FILD
n=37
P value
Underlying cause of death
Interstitial lung disease90 (84)61 (87)29 (78)0.238
 Cardiovascular disease8 (7.5)4 (5.7)4 (11)0.444
 Lung cancer2 (1.9)2 (2.9)00.543
 Other cancer4 (3.7)2 (2.9)2 (5.4)0.608
 Other reason*3 (2.8)1 (1.4)2 (5.4)0.274
Immediate cause of death
 Interstitial lung disease48 (45)34 (49)14 (38)0.288
 Lower respiratory tract infection31 (29)21 (30)10 (27)0.747
 Acute exacerbation of FILD or ARDS†12 (11)7 (10)5 (14)0.748
 Ischaemic heart disease7 (6.5)4 (5.7)3 (8.1)0.691
 Heart failure1 (0.9)01 (2.7)0.346
 Lung cancer2 (1.9)2 (2.9)00.543
 Other cancer2 (1.9)1 (1.4)1 (2.7)1.000
 Other infection1 (0.9)01 (2.7)0.346
 Pulmonary embolism1 (0.9)01 (2.7)0.346
 Other‡2 (1.9)1 (1.4)1 (2.7)1.000
  • *Drowning (non-specific interstitial pneumonia (NSIP)), morbus Alzheimer (IPF), Parkinson’s disease (unclassifiable FILD).

  • †While there is not a specific International Classification of Diseases version 10 code for acute exacerbation of fibrosing interstitial lung disease (AE-FILD), the immediate cause of death was classified as an AE-FILD, if this was clearly stated by the clinician who had signed the death certificate.

  • ‡Morbus Alzheimer (IPF), acute renal failure (NSIP).

  • ARDS, acute respiratory distress syndrome.