Clinical, radiologic, and physiologic features of idiopathic pulmonary fibrosis (IPF) with and without emphysema

ABSTRACT

Background

Idiopathic pulmonary fibrosis (IPF) can combine with emphysema, a condition termed as IPF with emphysema (IPFE). We compared the clinical, radiologic, and physiologic features of IPF and IPFE.

Research design and methods

Newly diagnosed IPF    and IPFE    patients were recruited between January 2018 and September 2020. Symptoms, high resolution computed tomography (HRCT), pulmonary function test (PFT) data, composite physiologic index (CPI), gender-age-physiology (GAP) scores, and follow-up data were obtained.

Results

The IPFE group had greater proportion of male smokers, and of lung cancer cases. The IPFE group had higher VC, FVC FEV₁, and lower FEV₁/FVC and DL_CO and lower percent fibrosis on HRCT. Both groups had similar symptoms and mortality. Mortality rate was associated with inability to perform PFT, CPI, GAP scores, percent fibrosis, VC, FVC, FEV_1, and DL_CO, serum SCC-Ag and CA125, and anti-fibrotic therapy (≥12 months) in IPF, while it was associated with inability to perform PFT, CPI, percent fibrosis, DL_CO, serum CEA, CYFRA21-1 and CA125, and anti-fibrotic therapy (≥12 months) in IPFE.

Conclusion

IPF and IPFE patients are different in smoking history, physiologic indices, HRCT patterns and prognostic factors, however, they have similar mortality. Anti-fibrotic therapy could improve the survival rate in both IPF and IPFE.

KEYWORDS:

• Idiopathic pulmonary fibrosis
• idiopathic pulmonary fibrosis with emphysema
• clinical features
• radiological pattern
• pulmonary function
• mortality

Acknowledgments

We would like to thank Prof. Kian Fan Chung of Imperial College London, National Heart&Lung Institute for his critical review of the manuscript.

Author contributions

F Li and H Yu conceived, designed and coordinated this research, and finalized the manuscript. C Li, Y Wang, and Q Liu collected and analyzed the data, and drafted the manuscript. H Zhang, F Xu, Z Gao, X Wang, Y Chen, and W Rong participated in collecting and analyzing of the data, and writing of the manuscript. G Tao and H Yu performed CT scanning and CT image analysis. All authors agree for the final version of the manuscript to be published.

Disclosure statement

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/17476348.2022.2093717

Additional information

Funding

This study was funded by National Key Technologies R & D Program Precision Medicine Research (2016YFC0901101 and 2021YFC2500700).