Long-term follow-up results of patients with combined pulmonary fibrosis and emphysema: A single-center experience
Respiratory Medicine, cilt.255, 2026 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 255
- Basım Tarihi: 2026
- Doi Numarası: 10.1016/j.rmed.2026.108780
- Dergi Adı: Respiratory Medicine
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE, Health Research Premium Collection (ProQuest)
- Akdeniz Üniversitesi Adresli: Evet
Özet
BackgroundCombined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical syndrome characterized by the coexistence of upper-lobe emphysema and lower-lobe fibrosis, most commonly in male smokers. Despite increasing recognition, long-term outcomes and prognostic determinants remain incompletely understood. The aim of this study is to evaluate long-term follow-up results of patients diagnosed with CPFE.Patients and methodBetween January 2015 and December 2024, a total of 588 patients with suspected CPFE were screened. Patients with at least one year of follow-up including radiological and physiological parameters were included in the study. Demographic data, comorbidities, pulmonary function tests, diffusing capacity for carbon monoxide (DLCO), 6-min walk test, echocardiographic findings, radiological progression, treatment strategies, and survival outcomes were analyzed. Emphysema severity was assessed using lobar scoring, and fibrotic patterns were classified according to Fleischner Society Criteria.ResultsThe final study population comprised 118 patients. The cohort was predominantly male (89.8%) with a mean age of 70 years, and 95.8% were current or former smokers. The median follow-up duration was 38 ± 6 months (range, 12–96 months). Radiological progression was observed in 67.8% of patients. Baseline and follow-up forced vital capacity (FVC) (%) values failed to predict progression, while DLCO decline at year 1 was significantly more frequent in patients with progression (p = 0.039). Regarding treatment, 36 patients (30.5%) received pirfenidone, six patients (5.1%) were treated with nintedanib, four patients (3.4%) received systemic corticosteroids, and one patient (0.8%) was treated with a biological agent. During the follow-up period, 38 patients died, while 80 survived. The mean overall survival was 5.61 ± 0.6 years, with a 5-year survival rate of 67.8%. Severe emphysema, acute exacerbation, 6-min walk test desaturation, lower values of FVC and DLCO, and higher pulmonary artery pressures were more significant in non-survivors. Cox regression analysis demonstrated that baseline DLCO (%) was an independent predictor of mortality (HR = 0.934, 95% CI: 0.902–0.966, p < 0.001).ConclusionCPFE has a tendency to progress, and that FVC alone is not a reliable tool for disease monitoring. The majority of patients are undertreated, and the long-term prognosis is generally poor. These findings emphasise the necessity for multidimensional assessment strategies in CPFE management.