Pathogenesis of IPF

A Fibrotic Theory of IPF Pathogenesis

  • Although the pathogenesis of IPF is not well understood. the primary theory suggests fibroproliferative processes1-4
    • Dysregulated wound healing occurs in the alveoli in response to an injury of unknown cause
    • Profibrotic signaling pathways are turned on and accelerate profibrotic processes, leading to an accumulation of extracellular matrix and fibrotic tissue that permanently and irreversibly alters lung architecture

Histopathologic Image Demonstrating the Fibrosis Present in the Lung of a Patient With IPF

Region of chronic fibrosis
Fibroblast focus

Figure 6. Image adapted from Raghu, et al. Am J Respir Crit Care Med. 2011;183:788-824.

Illustration of the Abnormal Wound-Healing Process in Idiopathic Pulmonary Fibrosis

Figure 7. Image adapted from Funke and Geiser. Swiss Med Wkly. 2015;145:w14139; and Selman, et al. Ann Intern Med. 2001;134:136-151.

Perspectives on IPF Pathogenesis

Nina Patel, MD, discusses the current perspectives on IPF pathogenesis in greater depth

Nina Patel, MD

Co-director of the Interstitial Lung Disease Program
Columbia University Medical Center

00:45
References

1. Raghu, et al. Am J Respir Crit Care Med. 2011;183:788-824. 2. Selman, et al. Ann Intern Med. 2001;134:136-151. 3. Wolters, et al. Annu Rev Pathol. 2014;9:157-179. 4. Funke and Geiser. Swiss Med Wkly. 2015;145:w14139.

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