The current classification of ILDs focuses on identifying the underlying cause because this frequently guides management decisions. In the setting of PFFs, determining a specific diagnosis within this group of diseases (disease splitting) is important early in patient management. This is because the early phase of PPF is specific to each disease subtype. On the other hand, due to similar clinical behavior and common underlying pathogenic mechanisms, treatment of PPF may be "lumped" together in late stages, when the diseases share a self-perpetuating fibrosis mechanism.
The distinction between IPF and non-IPF ILD is particularly important because immunotherapy often used in patients with non-IPF ILD is harmful for patients with IPF.
Learn more about the treatment of PF-ILD.
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Cite this: Zab Mosenifar. Fast Five Quiz: Interstitial Lung Disease Progressive Fibrosing Management - Medscape - Mar 06, 2023.