Shan Peng,1 Fanqing Meng,1 Cheng Wu,2 Mengshu Cao,3 Wei Li,1 Xiaoyan Xin,4 Anning Feng1 

1Department of Pathology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, People’s Republic of China; 2The Comprehensive Cancer Centre of Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, People’s Republic of China; 3Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, People’s Republic of China; 4Department of Radiology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, People’s Republic of China

Correspondence: Anning Feng, Department of Pathology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, 210008, People’s Republic of China, Email fengyee911@163.com

Abstract: Emphysema is common in fibrotic interstitial lung diseases, and its combination with pulmonary fibrosis is known as “Combined Pulmonary Fibrosis and Emphysema (CPFE) syndrome”. The diagnosis of CPFE significantly impacts treatment strategies and prognosis. In this article, we report the clinical, imaging, and especially the pathological features of two CPFE patients. Case 1: A 51-year-old male patient with a history of smoking. CT scans revealed interstitial lung disease combined with pulmonary bullae. Pathology showed extensive deposition of mononuclear cells in the alveolar spaces, with some cells phagocytosing pigment. Mild fibrous tissue hyperplasia was present in the lung interstitium, along with chronic inflammation and lymphoid nodule formation. The histological findings were consistent with desquamative interstitial pneumonia (DIP), and the clinical, imaging, and pathological correlation confirmed a diagnosis of CPFE. Case 2: A 58-year-old male, a driver with a history of dust exposure and smoking, was admitted due to chest tightness and a cough for 2 years. Chest CT revealed interstitial changes, emphysema, and bullae in both lungs. Histopathology showed fibrous widening of alveolar septa, mild chronic inflammation, and dust cell deposition, along with emphysematous changes and bulla formation, consistent with CPFE. The purpose of this report is to increase pathologists’ awareness of this complex disease and emphasize the importance of multidisciplinary cooperation in the diagnosis and treatment of CPFE. Furthermore, this article encourages further research into CPFE.

Keywords: CPFE, pulmonary fibrosis, emphysema, pathological diagnosis, multidisciplinary cooperation in diagnosis and treatment