Are antifibrotic treatment responses different in ıdiopathic pulmonary fibrosis patients with and without combined pulmonary fibrosis and emphysema?

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Objective: CPFE is a syndrome characterized by the coexistence of emphysema in the upper lung zones and fibrosis in the lower lung zones. This study compared the functional characteristics and mortality of IPF patients with and without emphysema following antifibrotic treatment. Methods: IPF patients who received at least six months of antifibrotic treatment between 2015 and 2022 were categorized into two groups based on the presence of emphysema. Pre- and post-treatment pulmonary function tests, clinical outcomes (emergency visits, hospital and ICU admissions), and mortality rates were analyzed. Results: Of the 204 IPF patients, 90 (44%) had CPFE. Following antifibrotic treatment, FEV1% and FVC% remained unchanged, while DLCO% declined in both groups (p<0.05). Functional parameter changes, emergency visits, hospital and ICU admissions, and mortality rates were similar between the groups. In Cox regression analysis, CPFE diagnosis (OR: 1.73, 95% CI: 1.06-2.83), low FVC% (OR: 0.97, 95% CI: 0.96-0.98), and device use (OR: 2.48, 95% CI: 1.50-4.09) were identified as independent risk factors for mortality. The five-year survival rate was 57%, and although CPFE patients had shorter survival, the difference was not statistically significant (p>0.05). Conclusion: Functional and clinical outcomes after antifibrotic treatment were similar in CPFE and IPF patients. While a decrease in DLCO% was observed, it remained within the acceptable range for treatment continuation. CPFE diagnosis, low FVC%, and device use were determined to be independent risk factors for mortality.

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