Progressive Pulmonary Fibrosis

 

Diagnostic Criteria for PPF:

To diagnose PPF, all three of the following must be present:

Underlying fibrosing ILD (non-IPF), with imaging or histology showing fibrosis.

Evidence of progression within the past year, based on at least one of the following:

1.Worsening respiratory symptoms, such as increasing dyspnea or cough.

2.Decline in lung function, especially:

Absolute decline in FVC ≥ 5% predicted

Decline in DLCO (diffusing capacity for carbon monoxide) ≥ 10% predicted

3.Progressive fibrosis on imaging, shown by:

Increased reticulation

New ground-glass opacities with traction bronchiectasis

Increased honeycombing

No alternative explanation (e.g., infection, heart failure, pulmonary embolism).