Table 3

Previous publications which have examined an acute respiratory worsening of idiopathic pulmonary fibrosis (IPF) and fibrosing interstitial lung diseases (FILD)

PublicationCriteria for inclusionFILD type (n)Survival
Huie et al, 2010, USA6Hospitalisation caused by acute respiratory worsening (ARW)IPF (13)
CTD-ILD (11)
Other ILD (4)
1-year survival:
Total 14.8%
IPF 0%
Non-IPF 28.6%
Moua et al, 2016, USA7Hospitalisation caused by ARWIPF (100)
CTD-ILD (56) i-NSIP (15)
CHP (11)
Other ILD (38)
Mortality at 1 year after last hospitalisation:
IPF 87%
Non-IPF 71%
Gannon et al, 2018, USA8Treatment in intensive care unit due to acute respiratory failureIPF (15)
CTD-ILD (23)
Other ILD (36) uIIP (52)
1-year mortality:
IPF 87%
CTD-ILD 52% other ILD 89% uIIP 85%
(p=0.02 CTD-ILD vs uIIP)
Arai et al, 2016, Japan9Patients with IIP undergone bronchoalveolar lavage (patients with AE-ILD)IPF (29)
Other IIP ‘possible UIP’ (12)
Other IIP ‘inconsistent with UIP’ (5)
Median survival after AE-ILD or possible AE-ILD:
IPF 68 days
Possible UIP 13 days
Inconsistent with UIP 39 days (p=0.028)
Suzuki et al, 2019, Japan10Patients with AE-FILD (consecutive patients with ILD of one hospital)IPF (124)
NSIP (2)
CHP (7)
CTD-ILD (27) uIIP (33)
90-day mortality:
IPF 38%
Non-IPF 47%
Murohashi et al, 2019, Japan11Hospitalisation due to acute or subacute IIP or CTD-ILD treated with steroid pulse therapyIPF (17)
NSIP (18)
AIP (6)
COP (4)
Other IIP (7)
CTD-ILD (16)
3-month mortality:
IPF 35.3%
Other IIP 35.7%
CTD-ILD 18.8%
Usui et al, 2013, Japan12Consecutive patients with AE of FILDIPF (30)
NSIP (18)
FPF (2)
Other (1)
Overall survival:
30% at 90 days
No difference IPF versus non-IPF
Cao et al, 2019, Japan13Patients with AE-IPF or AE-CTD-ILD admitted to a hospitalIPF (107)
CTD-ILD (70)
Median survival:
AE-CTD-ILD 35±4.2 days, better than AE-IPF (log rank, p=0.029).
Enomoto et al, 2019, Japan14Patients with AE-IPF or AE-CTD-ILD treated in a hospitalIPF 37
3-month mortality rate 46.7% for AE-CTD-ILD. No statistically significant difference compared with AE-IPF.
  • AE-CTD-ILD, acute exacerbation of connective tissue disease-associated interstitial lung disease; AE-FILD, acute exacerbation of fibrosing interstitial lung disease; AE-ILD, acute exacerbation of interstitial lung disease; AE-IPF, acute exacerbation of idiopathic pulmonary fibrosis; AIP, acute interstitial pneumonia; CHP, chronic hypersensitivity pneumonitis; COP, cryptogenic organising pneumonia; CTD, connective tissue disease; FPF, familial pulmonary fibrosis; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; i-NSIP, idiopathic non-specific interstitial pneumonia; NSIP, non-specific interstitial pneumonia; uIIP, unclassified idiopathic interstitial pneumonia; UIP, usual interstitial pneumonia.