| Interstitial lung diseases (ILDs) encompass a wide range of conditions with variable disease courses and prognoses. |
| Several ILDs, beyond idiopathic pulmonary fibrosis (IPF), can exhibit a progressive fibrotic phenotype with similar outcomes to IPF, recently categorized as progressive pulmonary fibrosis (PPF). |
| The epidemiological magnitude of PPF needs to be better established. |
| Understanding disease progression is crucial for developing standardized therapeutic management. |
| Current evidence points towards the beneficial effects of antifibrotic drugs in PPF. |
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