Skill Checkup: A 59-Year-Old Man With Dyspnea on Exertion and a Nonproductive Cough

Zab Mosenifar, MD


April 15, 2022

CT honeycombing, seen in this patient, is characteristic of the PF-ILD phenotype and is associated with a high mortality rate similar to that of idiopathic pulmonary fibrosis (IPF). The idea of PF-ILD has been studied and applied primarily among patients with IPF, but there is an increasing focus on progressive fibrosis in other subtypes of patients on the wide spectrum of underlying ILD diagnoses. This pattern is not characteristic of non-PF-ILDs, such as respiratory bronchiolitis-interstitial lung disease, ARDS, or desquamative interstitial pneumonia.

In the setting of PF-ILDs, determining a specific diagnosis within this group of diseases (disease splitting) is important early in patient management. This is because the early phase of PF-ILD is specific to each disease subtype. On the other hand, owing to similar clinical behavior and common underlying pathogenic mechanisms, treatment of PF-ILDs may be "lumped" together in late stages, when the diseases share a self-perpetuating fibrosis mechanism.


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