The epidemiological study of combined pulmonary fibrosis and emphysema in a community-based lung cancer screening program_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

XU Fei ^1,2 , CHEN Qunhui ³ , ZHANG Hai ¹ , GAO Zhenyun ¹ , WANG Xiaohui ⁴ , CHEN Yuqing ¹ , ZHANG Yanwei ¹ , HAN Baohui ¹ ,  LI Feng ¹

1. Department of Pulmonary and Critical Care Medicine, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, P. R. China;
2. Department of Internal Medicine, Shanghai Electric Power Hospital, Shanghai 200050, P. R. China;
3. Department of Radiology, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, P. R. China;
4. Department of Pharmacy, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, P. R. China;

Corresponding?author：

LI Feng, Email: lifeng741@aliyun.com

Keywords：

Combined pulmonary fibrosis and emphysema; Lung cancer screening program; Prevalence

DOI：

10.7507/1671-6205.202105049

Video：

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Objective To study the prevalence of combined pulmonary fibrosis and emphysema (CPFE) in a community-based lung cancer screening program in Shanghai. Methods From June 2018 to July 2019, eligible participants who were assessed through a high-risk lung cancer questionnaire in Xuhui, Shanghai underwent low-dose computed tomography of the lungs. The suspected CPFE patients were invited to provide medical history and blood for analysis, and received high-resolution CT (HRCT) scanning for confirmation. Results Of the 15 cases of suspected CPFE from a total of 4478 participants in which 1704 males and 2774 females, 4 declined further examination and 11 received further examine. Eight subjects were confirmed as CPFE, and all were male, of whom two were ex-smokers and six were active smokers. These CPFE patients had cough, chest tightness and dyspnea. There were 3 cases of centrilobular emphysema, 2 cases of paraseptal emphysema, 1 case of panlobular emphysema and 2 cases of mixed emphysema. There were 2 cases of usual interstitial pneumonia, 3 cases of non-specific interstitial pneumonia, 2 cases of airspace enlargement with fibrosis and 1 case of unclassifiable smoking-related interstitial fibrosis. The KBILD scores were 61.7±7.5 and mMRC scores were 1.5±0.8. Serum Krebs von den Lungen-6 concentration was (380.75±212.05)U/mL. Lung function test showed normal or mild restrictive ventilatory function, and mild-moderate impairment in diffusion capacity. Conclusions The prevalence of CPFE is 1.79‰ in a community-based lung cancer screening population, and is 4.69‰ in male lung cancer screening population.

Citation： XU Fei, CHEN Qunhui, ZHANG Hai, GAO Zhenyun, WANG Xiaohui, CHEN Yuqing, ZHANG Yanwei, HAN Baohui, LI Feng. The epidemiological study of combined pulmonary fibrosis and emphysema in a community-based lung cancer screening program. Chinese Journal of Respiratory and Critical Care Medicine, 2021, 20(10): 709-714. doi: 10.7507/1671-6205.202105049 Copy

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2.	Wang Q, Takashima S, Wang JC, Zheng LM, Sone S. Prevalence of emphysema in individuals who underwent screening CT for lung cancer in Nagano prefecture of Japan. Respiration, 2001, 68(4): 352-356.
3.	Nalysnyk L, Cid-Ruzafa J, Rotella P, Esser D. Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. Eur Respir Rev, 2012, 21(126): 355-361.
4.	Fujimoto K, Kitaguchi Y, Kubo K, Honda T. Clinical analysis of chronic obstructive pulmonary disease phenotypes classified using high-resolution computed tomography. Respirology, 2006, 11(6): 731-740.
5.	Washko GR, Hunninghake GM, Fernandez IE, et al; COPDGene Investigators. Lung volumes and emphysema in smokers with interstitial lung abnormalities. N Engl J Med, 2011, 364(10): 897-906.
6.	Ryerson CJ, Hartman T, Elicker BM, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest, 2013, 144(1): 234-240.
7.	Yoon HY, Kim TH, Seo JB, et al. Effects of emphysema on physiological and prognostic characteristics of lung function in idiopathic pulmonary fibrosis. Respirology, 2019, 24(1): 55-62.
8.	Chae KJ, Jin GY, Han YM, et al. Prevalence and progression of combined pulmonary fibrosis and emphysema in asymptomatic smokers: a case-control study. Eur Radiol, 2015, 25(8): 2326-2334.
9.	Ciccarese F, Attinà D, Zompatori M. Combined pulmonary fibrosis and emphysema (CPFE): what radiologist should know. Radiol Med, 2016, 121(7): 564-572.
10.	Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med, 2008, 177(2): 190-194.
11.	Avila NA, Brantly M, Premkumar A, et al. Hermansky-Pudlak syndrome: radiography and CT of the chest compared with pulmonary function tests and genetic studies. AJR Am J Roentgenol, 2002, 179(4): 887-892.
12.	Bergin C, Müller N, Nichols DM, et al. The diagnosis of emphysema. A computed tomographic-pathologic correlation 1986, 133(4): 541-546. Am Rev Respir Dis, 1986, 133(4): 541-546.
13.	Lin H, Jiang S. Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone. J Thorac Dis, 2015, 7(4): 767-779.
14.	Kinoshita Y, Watanabe K, Ishii H, et al. Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema. Histopathology, 2019, 74(7): 1103-1108.
15.	Zhang L, Zhang C, Dong F, et al. Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis. BMC Pulm Med, 2016, 16(1): 137.
16.	Morse D, Rosas IO. Tobacco smoke-induced lung fibrosis and emphysema. Annu Rev Physiol, 2014, 76: 493-513.
17.	Kwiatkowska S. IPF and CPFE - the two different entities or two different presentations of the same disease?. Adv Respir Med, 2018, 86(1): 23-26.
18.	Arish N, Petukhov D, Wallach-Dayan SB. The role of telomerase and telomeres in interstitial lung diseases: from molecules to clinical implications. Int J Mol Sci, 2019, 20(12): 2996.
19.	Matsuoka S, Yamashiro T, Matsushita S, et al. Quantitative CT evaluation in patients with combined pulmonary fibrosis and emphysema: correlation with pulmonary function. Acad Radiol, 2015, 22(5): 626-631.
20.	. Oikonomou A, Mintzopoulou P, Tzouvelekis A, et al. Pulmonary fibrosis and emphysema: is the emphysema type associated with the pattern of fibrosis? World J Radiol, 2015, 7(9): 294-305.
21.	Xu L, Yan DR, Zhu SL, et al. KL-6 regulated the expression of HGF, collagen and myofibroblast differentiation. Eur Rev Med Pharmacol Sci, 2013, 17(22): 3073-3077.
22.	Xu L, Bian W, Gu XH, et al. Differing expression of cytokines and tumor markers in combined pulmonary fibrosis and emphysema compared to emphysema and pulmonary fibrosis. COPD, 2017, 14(2): 245-250.
23.	Graney BA, Fischer A. Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc, 2019, 16(5): 525-533.
24.	Fernandes L, Nasser M, Ahmad K, et al. Interstitial pneumonia with autoimmune features (IPAF). Front Med (Lausanne), 2019, 6: 209.
25.	Alsumrain M, De Giacomi F, Nasim F, et al. Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival. Respir Med, 2019, 146: 106-112.
26.	Amariei DE, Dodia N, Deepak J, et al. Combined pulmonary fibrosis and emphysema: pulmonary function testing and a pathophysiology perspective. Medicina (Kaunas), 2019, 55(9): 580.

1. Cottin V, Nunes H, Brillet PY, et al; Groupe d'Etude et de Recherche sur les Maladies Orphelines Pulmonaires (GERM O P). Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J, 2005, 26(4): 586-593.
2. Wang Q, Takashima S, Wang JC, Zheng LM, Sone S. Prevalence of emphysema in individuals who underwent screening CT for lung cancer in Nagano prefecture of Japan. Respiration, 2001, 68(4): 352-356.
3. Nalysnyk L, Cid-Ruzafa J, Rotella P, Esser D. Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. Eur Respir Rev, 2012, 21(126): 355-361.
4. Fujimoto K, Kitaguchi Y, Kubo K, Honda T. Clinical analysis of chronic obstructive pulmonary disease phenotypes classified using high-resolution computed tomography. Respirology, 2006, 11(6): 731-740.
5. Washko GR, Hunninghake GM, Fernandez IE, et al; COPDGene Investigators. Lung volumes and emphysema in smokers with interstitial lung abnormalities. N Engl J Med, 2011, 364(10): 897-906.
6. Ryerson CJ, Hartman T, Elicker BM, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest, 2013, 144(1): 234-240.
7. Yoon HY, Kim TH, Seo JB, et al. Effects of emphysema on physiological and prognostic characteristics of lung function in idiopathic pulmonary fibrosis. Respirology, 2019, 24(1): 55-62.
8. Chae KJ, Jin GY, Han YM, et al. Prevalence and progression of combined pulmonary fibrosis and emphysema in asymptomatic smokers: a case-control study. Eur Radiol, 2015, 25(8): 2326-2334.
9. Ciccarese F, Attinà D, Zompatori M. Combined pulmonary fibrosis and emphysema (CPFE): what radiologist should know. Radiol Med, 2016, 121(7): 564-572.
10. Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med, 2008, 177(2): 190-194.
11. Avila NA, Brantly M, Premkumar A, et al. Hermansky-Pudlak syndrome: radiography and CT of the chest compared with pulmonary function tests and genetic studies. AJR Am J Roentgenol, 2002, 179(4): 887-892.
12. Bergin C, Müller N, Nichols DM, et al. The diagnosis of emphysema. A computed tomographic-pathologic correlation 1986, 133(4): 541-546. Am Rev Respir Dis, 1986, 133(4): 541-546.
13. Lin H, Jiang S. Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone. J Thorac Dis, 2015, 7(4): 767-779.
14. Kinoshita Y, Watanabe K, Ishii H, et al. Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema. Histopathology, 2019, 74(7): 1103-1108.
15. Zhang L, Zhang C, Dong F, et al. Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis. BMC Pulm Med, 2016, 16(1): 137.
16. Morse D, Rosas IO. Tobacco smoke-induced lung fibrosis and emphysema. Annu Rev Physiol, 2014, 76: 493-513.
17. Kwiatkowska S. IPF and CPFE - the two different entities or two different presentations of the same disease?. Adv Respir Med, 2018, 86(1): 23-26.
18. Arish N, Petukhov D, Wallach-Dayan SB. The role of telomerase and telomeres in interstitial lung diseases: from molecules to clinical implications. Int J Mol Sci, 2019, 20(12): 2996.
19. Matsuoka S, Yamashiro T, Matsushita S, et al. Quantitative CT evaluation in patients with combined pulmonary fibrosis and emphysema: correlation with pulmonary function. Acad Radiol, 2015, 22(5): 626-631.
20. . Oikonomou A, Mintzopoulou P, Tzouvelekis A, et al. Pulmonary fibrosis and emphysema: is the emphysema type associated with the pattern of fibrosis? World J Radiol, 2015, 7(9): 294-305.
21. Xu L, Yan DR, Zhu SL, et al. KL-6 regulated the expression of HGF, collagen and myofibroblast differentiation. Eur Rev Med Pharmacol Sci, 2013, 17(22): 3073-3077.
22. Xu L, Bian W, Gu XH, et al. Differing expression of cytokines and tumor markers in combined pulmonary fibrosis and emphysema compared to emphysema and pulmonary fibrosis. COPD, 2017, 14(2): 245-250.
23. Graney BA, Fischer A. Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc, 2019, 16(5): 525-533.
24. Fernandes L, Nasser M, Ahmad K, et al. Interstitial pneumonia with autoimmune features (IPAF). Front Med (Lausanne), 2019, 6: 209.
25. Alsumrain M, De Giacomi F, Nasim F, et al. Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival. Respir Med, 2019, 146: 106-112.
26. Amariei DE, Dodia N, Deepak J, et al. Combined pulmonary fibrosis and emphysema: pulmonary function testing and a pathophysiology perspective. Medicina (Kaunas), 2019, 55(9): 580.

Chinese Journal of Respiratory and Critical Care Medicine

The epidemiological study of combined pulmonary fibrosis and emphysema in a community-based lung cancer screening program

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