HR* | 95% CI | P value | |
Stable state | |||
Sex, male/female | 0.570 | 0.225 to 1.442 | 0.235 |
Smoking, y/n | 0.570 | 0.235 to 1.442 | 0.235 |
Diagnosis, IPF/non-IPF | 0.966 | 0.434 to 2.151 | 0.932 |
Honeycomb on HRCT, y/n | 1.234 | 0.548 to 2.779 | 0.621 |
mMRC, ≥2/≤1 | 1.456 | 0.623 to 3.403 | 0.386 |
Disease severity, IV/I-III | 1.500 | 0.641 to 3.508 | 0.350 |
LTOT, y/n | 1.453 | 0.645 to 3.274 | 0.367 |
Prednisolone, y/n | 2.560 | 1.143 to 5.732 | 0.022 |
Immunosuppressant, y/n | 2.204 | 0.821 to 5.920 | 0.117 |
Pirfenidone, y/n | 1.514 | 0.450 to 5.085 | 0.503 |
Nintedanib, y/n | 0.705 | 0.095 to 5.233 | 0.733 |
Antifibrotic drugs, y/n† | 1.086 | 0.323 to 3.647 | 0.894 |
At the onset of AE | |||
Age, years | 1.014 | 0.968 to 1.063 | 0.545 |
Body weight, kg | 1.021 | 0.987 to 1.056 | 0.230 |
HRCT pattern, diffuse vs non-diffuse | 1.623 | 0.728 to 3.621 | 0.237 |
PaO2/FiO2 ratio | 0.997 | 0.993 to 1.001 | 0.114 |
PaO2/FiO2 ratio, ≤200 vs >200 | 2.659 | 1.099 to 6.433 | 0.030 |
WBC, /μL | 1.000 | 1.000 to 1.000 | 0.138 |
LDH, U/mL | 1.002 | 0.998 to 1.005 | 0.276 |
KL-6, x100 U/mL | 1.018 | 0.976 to 1.061 | 0.408 |
CRP, mg/dL | 1.024 | 0.975 to 1.075 | 0.350 |
DIC, y/n | 0.864 | 0.203 to 3.677 | 0.843 |
FDP/d-dimer, lower/higher‡ | 4.746 | 2.072 to 10.870 | <0.001 |
Treatment for AE-IIPs | |||
NPPV or IPPV, y/n | 1.841 | 0.815 to 4.155 | 0.142 |
Macrolide, y/n | 0.841 | 0.314 to 2.254 | 0.731 |
Immunosuppressant, y/n | 1.283 | 0.549 to 3.001 | 0.565 |
PMX-DHP, y/n | 2.867 | 0.850 to 9.672 | 0.090 |
Anticoagulants, y/n | 1.772 | 0.756 to 4.157 | 0.188 |
Antiplatelet drugs, y/n | 0.531 | 0.181 to 1.558 | 0.249 |
*HR >1 indicates worse survival.
†Nintedanib and/or pirfenidone.
‡Higher FDP is 10 mg/L≤FDP and lower FDP is <10 mg/L as described in the methodology.
AE, acute exacerbation; CRP, C-reactive protein; DIC, disseminated intravascular coagulation; FDP, fibrin degradation product; FiO2, fraction of inspired oxygen; HRCT, high-resolution CT; IIP, idiopathic interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; IPPV, invasive positive pressure ventilation; KL-6, Krebs von den Lungen-6; LDH, lactate dehydrogenase; LTOT, long-term oxygen therapy; mMRC, modified Medical Research Council scores; NPPV, non-invasive positive pressure ventilation; PaO2, partial pressure of oxygen; PMX-DHP, direct haemoperfusion with polymyxin B-immobilisedfibre column; WBC, white blood cell counts; y/n, yes/no.