Episode Details

In this APAPP FAQ, Jessica Glennie, CNP, discusses what drives fibrotic disease progression in idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). Jessica explains how repeated microinjuries to the alveolar epithelial cells can disrupt the lungs’ natural repair process, triggering the release of profibrotic growth factors and cytokines. These signals recruit fibroblasts, promote their transformation into myofibroblasts, and lead to excessive collagen deposition and extracellular matrix production.

This video reviews the abnormal tissue and vascular remodeling that contributes to progressive lung scarring, reduced lung function, and potential pulmonary hypertension. Jessica also explains key differences between IPF and PPF, including the role inflammation may play in some forms of PPF, and discusses genetic factors associated with pulmonary fibrosis, including short telomeres and mutations involving TERT, TERC, MUC5B, and TOLLIP. Ideal for pulmonology providers, interstitial lung disease specialists, and advanced practice providers, this FAQ provides a concise overview of the biological and genetic mechanisms that contribute to pulmonary fibrosis progression.

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