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Etiology of Idiopathic Pulmonary Fibrosis

Determine the underlying etiology (causal mechanisms and initiating factors) of idiopathic pulmonary fibrosis (IPF), a chronic, progressive interstitial lung disease whose cause remains unknown, in order to clarify disease initiation and guide prevention and therapeutic strategies.

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Background

The paper introduces IPF as a severe interstitial lung disease with poor outcomes and notes that despite extensive research, the initiating cause of IPF is still not identified. The authors frame their paper within broader efforts to understand fibrosis progression, while acknowledging that the root cause of IPF remains unknown.

Clarifying the etiology of IPF would provide foundational insight into why fibrotic remodeling begins and persists, complementing mechanistic studies like those presented here that focus on fibroblast-to-myofibroblast transitions and intercellular mechanical signaling.

References

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive interstitial lung disease of unknown etiology 1,2.

Intercellular contact is sufficient to drive Fibroblast to Myofibroblast transitions (2503.01834 - Chandar et al., 3 Mar 2025) in Introduction, first paragraph