Histopathological UIP | Hospitalisation for respiratory decline, pneumothorax or acute exacerbation |
Radiographic probable or definite UIP pattern | Desaturation to <88% on 6MWT or >50 m decline in 6MWD over 6 months |
FVC <80% or DLCO <40% pred | Pulmonary hypertension on right heart catheterisation or echocardiography |
Relative decline in pulmonary function over the past 2 years: FVC ≥10% or DLCO ≥15% or FVC ≥5% with symptomatic or radiographic progression | Absolute decline in pulmonary function over the past 6 months despite appropriate treatment: FVC >10% or DLCO >10% or FVC >5% with radiographic progression |
Any resting or exertional oxygen requirement | |
For inflammatory ILDs, disease progression despite treatment | |