A retrospective study of 61 patients with combined pulmonary fibrosis and emphysema (CPFE) identified a distinct clinical entity characterized by subnormal spirometry, severe gas exchange impairment, high prevalence of pulmonary hypertension, and poor survival. All patients were smokers, with a mean age of 65 years. Dyspnea on exertion was present in all, and 87% had basal crackles. Pulmonary function tests showed reduced lung volumes and impaired gas exchange. Pulmonary hypertension was present in 47% at diagnosis and 55% during follow-up. Survival was 87.5% at 2 years and 54.6% at 5 years, with a median survival of 6.1 years. Pulmonary hypertension at diagnosis was a critical determinant of prognosis. CT scans showed emphysema in the upper zones and fibrosis in the lower zones. The CT pattern was typical of idiopathic pulmonary fibrosis (IPF) in 31 patients and strongly suggestive of IPF in 21. Histological analysis confirmed centrilobular emphysema and interstitial pneumonia. Treatment included corticosteroids and oxygen therapy, but outcomes were poor. Survival was worse than in IPF but better than in emphysema alone. Pulmonary hypertension was common in CPFE, higher than in IPF or COPD. The study highlights the need for recognition of CPFE as a distinct entity, with pulmonary hypertension being a critical prognostic factor. The syndrome is underrecognized due to its overlap with IPF and emphysema. The study provides the first survival analysis of CPFE, showing a median survival of 6.1 years. The presence of pulmonary hypertension at diagnosis significantly affects prognosis. The study suggests that CPFE may result from a combination of smoking-related interstitial lung disease and emphysema. Therapeutic options are limited, and specific treatments for pulmonary hypertension are not yet evaluated. The syndrome is characterized by preserved lung volumes, impaired gas exchange, and exercise-induced hypoxemia. The study emphasizes the importance of accurate diagnosis and management of CPFE.A retrospective study of 61 patients with combined pulmonary fibrosis and emphysema (CPFE) identified a distinct clinical entity characterized by subnormal spirometry, severe gas exchange impairment, high prevalence of pulmonary hypertension, and poor survival. All patients were smokers, with a mean age of 65 years. Dyspnea on exertion was present in all, and 87% had basal crackles. Pulmonary function tests showed reduced lung volumes and impaired gas exchange. Pulmonary hypertension was present in 47% at diagnosis and 55% during follow-up. Survival was 87.5% at 2 years and 54.6% at 5 years, with a median survival of 6.1 years. Pulmonary hypertension at diagnosis was a critical determinant of prognosis. CT scans showed emphysema in the upper zones and fibrosis in the lower zones. The CT pattern was typical of idiopathic pulmonary fibrosis (IPF) in 31 patients and strongly suggestive of IPF in 21. Histological analysis confirmed centrilobular emphysema and interstitial pneumonia. Treatment included corticosteroids and oxygen therapy, but outcomes were poor. Survival was worse than in IPF but better than in emphysema alone. Pulmonary hypertension was common in CPFE, higher than in IPF or COPD. The study highlights the need for recognition of CPFE as a distinct entity, with pulmonary hypertension being a critical prognostic factor. The syndrome is underrecognized due to its overlap with IPF and emphysema. The study provides the first survival analysis of CPFE, showing a median survival of 6.1 years. The presence of pulmonary hypertension at diagnosis significantly affects prognosis. The study suggests that CPFE may result from a combination of smoking-related interstitial lung disease and emphysema. Therapeutic options are limited, and specific treatments for pulmonary hypertension are not yet evaluated. The syndrome is characterized by preserved lung volumes, impaired gas exchange, and exercise-induced hypoxemia. The study emphasizes the importance of accurate diagnosis and management of CPFE.