Comparative Analysis of Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis: Epidemiology, Pathophysiology, Clinical Features, Diagnosis and Treatment

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial disease of unknown origin, characterized by radiological and histological features consistent with usual interstitial pneumonia (UIP). It is marked by a progressive worsening of dyspnea and a decline in lung function. Both IPF and PPF are comparable because they have poor prognoses with a median survival time from diagnosis of around 2-4 years without antifibrotic therapy. This review shows the main specific characteristics and differences of epidemiology, pathophysiology, clinical and radiological features, treatment, and prognosis of IPF and PPF.

Keywords

Idiopathic pulmonary fibrosis / Progressive pulmonary fibrosis / Fibrosis interstitial pneumonia / Interstitial lung disease

Acknowledgments

We extend our gratitude to the patients and their families for their valuable contributions to advancing the understanding and management of idiopathic and progressive pulmonary fibrosis.

Author Contributions

C.M.D., J.S.T. and E.C. contributed to the conception and design of the study. C.M.D. drafted the manuscript. J.S.T. and E.C. critically reviewed and revised the manuscript for intellectual content. All authors approved the final version of the manuscript.

Ethics Statement

Not applicable.

Informed Consent Statement

Not applicable.

Data Availability Statement

Not applicable.

Funding

The study has been financed by FEDER Funds (PI23/00294), SEPAR, SOCAP, FUCAP and the August Pi i Sunyer Biomedical Research Institute (IDIBAPS).

Declaration of Competing Interest

J.S.T. reports receiving grants and consulting fees from Boehringer, Aflofarm and Roche. C.M.D. and E.C. declare no competing interests.

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