Emphysema or fibrosis progression in patients with combined pulmonary fibrosis and emphysema

Background: Patients with combined pulmonary fibrosis and emphysema (CPFE) may experience emphysema or fibrosis progression on chest computed tomography (CT). This study aimed to investigate the relationship and prognosis in CPFE patients with emphysema or fibrosis progression.

Methods: A total of 188 CPFE patients were included in our retrospective cohort study. The clinical presentations, radiographic features, and laboratory findings of the patients were reviewed.

Results: Among CPFE patients, 28.1% exhibited emphysema progression and 43.3% showed fibrosis progression. Different higher tumour markers were observed in the emphysema or fibrosis progression groups. Smoking, definite usual interstitial pneumonia (UIP), and total extent of emphysema were risk factors for emphysema progression. Age, definite UIP, and mediastinal lymph node enlargement were risk factors for fibrosis progression. Patients with fibrosis progression had worse prognoses than patients without fibrosis progression (HR 2.159; 95%CI, 1.243-3.749; P = 0.006). However, the prognosis was similar between patients with and without emphysema progression (HR 0.839; 95%CI, 0.429-1.641; P = 0.608). There was no significant interaction between emphysema and fibrosis progression (p>0.05).

Conclusions: In CPFE patients, emphysema and fibrosis progression had different higher tumour markers, risk factors, and prognosis effects. There was no significant interaction between emphysema and fibrosis progression. Fibrosis progression had a deleterious effect on prognosis, whereas emphysema progression did not affect prognosis. Therefore, the primary objective of CPFE treatment should be to halt or even reverse the progression of fibrosis. CPFE may be primarily a fibrotic disease, with emphysema being an incidental complication.