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双重威胁:合并肺纤维化和肺气肿(CPFE):两例报告

The Dual Threat Combined Pulmonary Fibrosis and Emphysema (CPFE): Two Cases.

作者信息

Peng Shan, Meng Fanqing, Wu Cheng, Cao Mengshu, Li Wei, Xin Xiaoyan, Feng Anning

机构信息

Department of Pathology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, People's Republic of China.

The Comprehensive Cancer Centre of Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, People's Republic of China.

出版信息

Int J Chron Obstruct Pulmon Dis. 2025 Aug 15;20:2885-2891. doi: 10.2147/COPD.S523597. eCollection 2025.

DOI:10.2147/COPD.S523597
PMID:40837109
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12364000/
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. : 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. : 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.

摘要

肺气肿在纤维化间质性肺疾病中很常见,其与肺纤维化合并存在被称为“合并性肺纤维化和肺气肿(CPFE)综合征”。CPFE的诊断对治疗策略和预后有重大影响。在本文中,我们报告了两名CPFE患者的临床、影像学,尤其是病理特征。:一名51岁有吸烟史的男性患者。CT扫描显示间质性肺疾病合并肺大疱。病理显示肺泡腔内有大量单核细胞沉积,部分细胞吞噬色素。肺间质有轻度纤维组织增生,伴有慢性炎症和淋巴小结形成。组织学表现符合脱屑性间质性肺炎(DIP),临床、影像学和病理相关性证实为CPFE诊断。:一名58岁男性,司机,有粉尘接触史和吸烟史,因胸闷、咳嗽2年入院。胸部CT显示双肺间质改变、肺气肿和肺大疱。组织病理学显示肺泡间隔纤维增宽、轻度慢性炎症和尘细胞沉积,伴有肺气肿改变和肺大疱形成,符合CPFE。本报告的目的是提高病理学家对这种复杂疾病的认识,并强调多学科合作在CPFE诊断和治疗中的重要性。此外,本文鼓励对CPFE进行进一步研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/3a54e02dc7ff/COPD-20-2885-g0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/6dee282104da/COPD-20-2885-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/4f46a2d0049c/COPD-20-2885-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/8669f53046f0/COPD-20-2885-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/3a54e02dc7ff/COPD-20-2885-g0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/6dee282104da/COPD-20-2885-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/4f46a2d0049c/COPD-20-2885-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/8669f53046f0/COPD-20-2885-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0479/12364000/3a54e02dc7ff/COPD-20-2885-g0004.jpg

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本文引用的文献

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Curr Opin Pulm Med. 2024 Mar 1;30(2):167-173. doi: 10.1097/MCP.0000000000001044. Epub 2023 Dec 26.
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Int J Chron Obstruct Pulmon Dis. 2021 Jun 21;16:1873-1885. doi: 10.2147/COPD.S307192. eCollection 2021.
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