Publication | Criteria for inclusion | FILD type (n) | Survival |
Huie et al, 2010, USA6 | Hospitalisation 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% (p=0.045) |
Moua et al, 2016, USA7 | Hospitalisation caused by ARW | IPF (100) CTD-ILD (56) i-NSIP (15) CHP (11) Other ILD (38) | Mortality at 1 year after last hospitalisation: IPF 87% Non-IPF 71% (p=0.003) |
Gannon et al, 2018, USA8 | Treatment in intensive care unit due to acute respiratory failure | IPF (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, Japan9 | Patients 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, Japan10 | Patients 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% (p=0.345) |
Murohashi et al, 2019, Japan11 | Hospitalisation due to acute or subacute IIP or CTD-ILD treated with steroid pulse therapy | IPF (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% (p=0.460) |
Usui et al, 2013, Japan12 | Consecutive patients with AE of FILD | IPF (30) NSIP (18) FPF (2) Other (1) | Overall survival: 30% at 90 days No difference IPF versus non-IPF |
Cao et al, 2019, Japan13 | Patients with AE-IPF or AE-CTD-ILD admitted to a hospital | IPF (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, Japan14 | Patients with AE-IPF or AE-CTD-ILD treated in a hospital | IPF 37 CTD-ILD 15 | 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.