Combined Pulmonary Fibrosis and Emphysema: Current Status and Future Perspective

Abstract

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.