What is pulmonary fibrosis combined with emphysema? Pulmonary fibrosis with emphysema (CPFE) is a smoking-associated interstitial lung disease that is a specific phenotype of pulmonary fibrosis, manifested on chest imaging as bilateral upper lobe emphysema and bilateral lower lobe pulmonary fibrosis. Pulmonary fibrosis combined with emphysema can be seen in different etiologies of pulmonary fibrosis, with idiopathic pulmonary fibrosis combined with emphysema being more common. What are the clinical manifestations of pulmonary fibrosis combined with emphysema? Patients usually present with progressive shortness of breath and dyspnea, which worsens with activity and is accompanied by a dry cough. In severe cases, patients may have shortness of breath in a quiet state, inability to lie down at night, and edema of both lower limbs. How is pulmonary fibrosis combined with emphysema diagnosed? The diagnosis of pulmonary fibrosis combined with emphysema is considered when the smoker’s high resolution CT of the chest reveals signs of emphysema in both upper lung lobes, thickened interlobular septa and grid shadow in both middle and lower lung lobes, or even honeycomb lung, with or without distraction bronchodilation. Patients have a more specific lung function, with lung volumes usually remaining in the normal range, but with a significant decrease in diffusion volume and severe gas exchange impairment. Pulmonary hypertension is easily combined with hypoxemia. How is pulmonary fibrosis combined with emphysema treated? Patients with hypoxemia require home oxygen therapy to maintain transcutaneous oxygen saturation above 90% for greater than 15 hours per day, or even 24 hours per day in severe cases. Treatment is tailored to the different causes of pulmonary fibrosis, such as allergic pneumonia, avoidance of exposure factors, oral glucocorticoids and/or immunosuppressants, and idiopathic pulmonary fibrosis, oral anti-pulmonary fibrosis drugs, such as pirfenidone. Symptomatic treatment with cough suppression and expectoration. Patients with combined pulmonary hypertension are given pulmonary artery pressure-lowering and anti-heart failure therapy, etc. What is the prognosis for pulmonary fibrosis combined with emphysema? Patients usually have significant gas exchange problems, are prone to respiratory failure, and have a worse prognosis than those with pulmonary fibrosis or emphysema alone because of the combination of pulmonary hypertension.