Learning Theater

Through the Pulmonologist’s Lens: Understanding the Patient Experience in IPF and PPF

Learning Theater 2
Sponsored by Bristol Myers Squibb

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Program Description

Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are life-limiting interstitial lung diseases that present significant challenges for patients and clinicians, marked by diagnostic delays, management complexities, and persistent unmet needs.^1–3

This Learning Theater, sponsored by Bristol Myers Squibb, will discuss the challenges of managing the course of these conditions in clinical practice, linking the experience of patients to the pathophysiology of IPF and PPF by exploring the underlying biology and signaling pathways.

Compelling patient cases will trace the full IPF/PPF journey from early symptom onset and diagnostic hurdles to management approaches, highlighting the barriers patients face and the gaps that continue to affect outcomes and quality of life.

The discussion will then dive deeper into disease pathophysiology, exploring signaling pathways implicated in the three drivers of fibrogenesis (faulty epithelial cell repair, inflammation/vascular leakage, and fibroblast activation and expansion)^4,5, and how the LPA-LPA1 pathway acts as a central mediator that mechanistically links these processes in IPF and PPF.^6–9

Join us as we delve into the IPF and PPF patient experience through the pulmonologist’s lens and explore how our evolving understanding of the pathophysiology of these conditions may shape the future of care.

This is a non-CME event and does not qualify for CME or CE, or MOC credit. This event is not part of the official CHEST Annual Meeting 2026 conference sessions. This event is not an endorsement by CHEST and does not reflect the views or opinions of CHEST.

References

1. Hoyer N, Prior TS, Bendstrup E, Shaker SB. Diagnostic delay in IPF impacts progression-free survival, quality of life and hospitalisation rates. BMJ Open Respir Res. 2022;9(1):e001276. doi:10.1136/bmjresp-2022-001276
2. Moor CC, Wijsenbeek MS, Balestro E, et al. Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey. ERJ Open Res. 2019;5(4):00124-2019. Published 2019 Oct 21. doi:10.1183/23120541.00124-2019
3. Chaudhuri N, Spagnolo P, Valenzuela C, et al. Treatment patterns and patient journey in progressive pulmonary fibrosis: a cross-sectional survey. Respir Res. 2024;25(1):364. Published 2024 Oct 9. doi:10.1186/s12931-024-02995-9
4. Ma H, Liu S, Li S, Xia Y. Targeting Growth Factor and Cytokine Pathways to Treat Idiopathic Pulmonary Fibrosis. Front Pharmacol. 2022;13:918771. Published 2022 Jun 3. doi:10.3389/fphar.2022.918771
5. Probst CK, Montesi SB, Medoff BD, Shea BS, Knipe RS. Vascular permeability in the fibrotic lung. Eur Respir J. 2020;56(1):1900100. Published 2020 Jul 16. doi:10.1183/13993003.00100-2019
6. Volkmann ER, Denton CP, Kolb M, et al. Lysophosphatidic acid receptor 1 inhibition: a potential treatment target for pulmonary fibrosis. Eur Respir Rev. 2024;33(172):240015. Published 2024 Jun 12. doi:10.1183/16000617.0015-2024
7. Funke M, Zhao Z, Xu Y, Chun J, Tager AM. The lysophosphatidic acid receptor LPA1 promotes epithelial cell apoptosis after lung injury. Am J Respir Cell Mol Biol. 2012;46(3):355-364. doi:10.1165/rcmb.2010-0155OC
8. Zhao J, He D, Su Y, et al. Lysophosphatidic acid receptor 1 modulates lipopolysaccharide-induced inflammation in alveolar epithelial cells and murine lungs. Am J Physiol Lung Cell Mol Physiol. 2011;301(4):L547-L556. doi:10.1152/ajplung.00058.2011
9. Tager AM, LaCamera P, Shea BS, et al. The lysophosphatidic acid receptor LPA1 links pulmonary fibrosis to lung injury by mediating fibroblast recruitment and vascular leak. Nat Med. 2008;14(1):45-54. doi:10.1038/nm1685

Presenters

Scott Matson, MD
University of Kansas Health System – Medical Pavilion
Physician
Kansas City, KS