Does combined pulmonary fibrosis and emphysema syndrome affect response to antifibrotic therapy and survival? A single-center retrospective cohort study

dc.authorid0000-0003-2380-5410
dc.authorid0000-0001-9694-7909
dc.authorid0000-0002-6484-0039
dc.authorid0009-0001-7437-6583
dc.authorid0000-0002-1163-125X
dc.authorid0000-0001-9731-3190
dc.authorid0000-0001-9129-5497
dc.authorid0000-0002-2443-2639
dc.authorid0000-0001-9262-7078
dc.authorid0000-0002-3448-3675
dc.contributor.authorTeke, Nazlı Hüma
dc.contributor.authorAğca, Meltem
dc.contributor.authorTürkar, Ayla
dc.contributor.authorSevim, Tülin
dc.contributor.authorTuncay, Eylem
dc.contributor.authorGüngör, Sinem
dc.contributor.authorYıldırım, Elif
dc.contributor.authorÖzbaki, Fatma
dc.contributor.authorGündoğuş, Baran
dc.contributor.authorArınç, Sibel
dc.contributor.authorBerk Takır, Huriye
dc.contributor.authorÖzmen, İpek
dc.date.accessioned2026-01-24T20:36:18Z
dc.date.available2026-01-24T20:36:18Z
dc.date.issued2025
dc.departmentFakülteler, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Acil Tıp Ana Bilim Dalı
dc.description.abstractObjective: Combined pulmonary fibrosis and emphysema (CPFE) is a clinicoradiological syndrome characterized by upper lobe emphysema and lower lobe fibrosis, most commonly associated with idiopathic pulmonary fibrosis (IPF). This study compared the clinical characteristics, functional parameters, and mortality of patients with CPFE and IPF who received antifibrotic therapy. Methods: Between October 2015 and Au gust 2022, patients with IPF treated with antifibrotics for at least 6 months were retrospectively evaluated and divided into two groups: CPFE (emphysema present) and IPF (emphysema absent). Demographic data, antifi brotic therapy, functional parameters before and after treatment (FEV1%, FVC%, DLco %), clinical outcomes (hospital admissions, mortality) were compared. Results: Of the 204 patients with IPF, 90 (44%) had CPFE. CPFE patients were more often male, had greater smoking history, higher pack-years, and more lung cancer than IPF patients (p < 0.001 for all). Post-treatment FEV1% and FVC% did not significantly differ between the groups, whereas DLco% declined significantly in both (p < 0.001 and p = 0.002). DLco% decreased more in IPF than CPFE, but the difference was not statistically significant [−3 (−11–3) vs. −0.43 (−1.1–0.2), p = 0.36]. The hospital admission rates were similar. Independent risk factors for mortality included CPFE diagnosis (HR: 1.73, 95% CI: 1.06–2.83, p = 0.029), low FVC% (HR: 0.970, 95% CI: 0.96–0.98, p < 0.001), and device use (long-term oxygen therapy [LTOT] or home non-invasive mechanical ventilation [NIMV]) (HR: 2.48, 95% CI: 1.50–4.09, p < 0.001). Mean survival was shorter in patients with emphysema than in those without em physema (5.08 vs. 5.68 years, p = 0.08). Conclusions: Despite a decline in DLco%, changes remained below the futility threshold. Clinical outcomes and mortality were comparable. CPFE diagnosis, low FVC%, and LTOT/ NIMV use independently predicted higher mortality.
dc.identifier.citationTeke, N. H., Ağca, M., Türkar, A., Sevim, T., Tuncay, E., Güngör, S., Yıldırım, E., Özbaki, F., Gündoğuş, B., Arınç, S., Berk Takır, H., & Özmen, İ. (2025). Does combined pulmonary fibrosis and emphysema syndrome affect response to antifibrotic therapy and survival? A single-center retrospective cohort study. Sarcoidosis Vasculitis and Diffuse Lung Diseases, 42(4), pp. 1-11. https://doi.org/10.36141/svdld.v42i4.17187
dc.identifier.doi10.36141/svdld.v42i4.17187
dc.identifier.endpage11
dc.identifier.issn1124-0490
dc.identifier.issn2532-179X
dc.identifier.issue4
dc.identifier.pmidPMID: 41396111
dc.identifier.scopusqualityQ3
dc.identifier.startpage1
dc.identifier.urihttps://doi.org/10.36141/svdld.v42i4.17187
dc.identifier.urihttps://hdl.handle.net/20.500.13055/1274
dc.identifier.volume42
dc.identifier.wosqualityQ3
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.indekslendigikaynak.otherSCI-E - Science Citation Index Expanded
dc.institutionauthorSevim, Tülin
dc.institutionauthorid0009-0001-7437-6583
dc.language.isoen
dc.publisherMattioli 1885
dc.relation.ispartofSarcoidosis Vasculitis and Diffuse Lung Diseases
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectAntifibrotic Treatment
dc.subjectCombined Pulmonary Fibrosis and Emphysema
dc.subjectIdiopathic Pulmonary Fibrosis
dc.titleDoes combined pulmonary fibrosis and emphysema syndrome affect response to antifibrotic therapy and survival? A single-center retrospective cohort study
dc.typeArticle
dspace.entity.typePublication

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