Year: 2026
Type: review
Abstract: BACKGROUND: Combined pulmonary fibrosis and emphysema (CPFE) is a clinical syndrome defined by the coexistence of upper-lobe emphysema and lower-lobe pulmonary fibrosis. It is characterized by clinical, functional, and radiologic features that differ significantly from isolated idiopathic pulmonary fibrosis or chronic obstructive pulmonary disease. SUMMARY: This review summarizes recent advances in CPFE, focusing on etiologic classification, clinical and radiologic features, diagnosis and treatment, prognostic evaluation, and emerging perspectives in artificial intelligence (AI). Based on current research, we summarize the etiologic classification of CPFE into four types: smoking-related, systemic autoimmune rheumatic disease-associated, occupational- and environmental-related, and idiopathic. These types differ in epidemiologic characteristics, imaging patterns, and disease progression. Consequently, future research is expected to focus on mechanism-driven phenotyping and quantitative imaging analysis, where AI may provide additional value in disease identification, risk stratification, and dynamic prognostic assessment. KEY MESSAGE: This review proposes a novel framework for CPFE, integrating etiologic subtyping and a diagnostic algorithm to guide precise management, and highlights AI-powered strategies for future personalized interventions.
Source: The Clinical Respiratory Journal
DOI: 10.1111/crj.70236
Institutions: Shanghai Jiao Tong University, Shanghai Chest Hospital
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,310
Awards: G4804211158, G5888408915
OpenAlex ID: W7225223419