Clinical characteristics and risk factors of combined pulmonary fibrosis and emphysema syndrome_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

WU Suji ¹ , WEI Yi ² , SHI Junqing ³ , ZHANG Xu ³ ,  XIE Min ¹

1. Department of Respiratory and Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Central Transportation, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P. R. China;
3. Department of Respiratory and Critical Care Medicine, Chengdu Second People’s hospital, Chengdu, Sichuan 610017, P. R. China;

Corresponding author：

XIE Min, Email: xieminhuaxi@163.com

Keywords：

Combined pulmonary fibrosis and emphysema; Risk factors; Pulmonary hypertension; Lung cancer

DOI：

10.7507/1671-6205.201909048

Video：

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Objective Based on real-word data, and compared with two common chronic respiratory diseases, interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD), this case-control study plans to investigate the risk factors and clinical characteristics of patients with combined pulmonary fibrosis and emphysema syndrome (CPFE).Methods A retrospective case-control study was carried out to screen the clinical data of 96 patients with CPFE, 133 patients with COPD and 164 patients with ILD, analyze their demographics, clinical data, complications and related clinical indicators. Univariate analysis was used to compare the differences among the three groups, and multivariate logistic analysis was used to screen for risk factors.Results All three groups were in old age with the average age of above 71 years. In terms of male ratio and smoking rate, the CPFE group (93.8%, 85.4%) was higher than the ILD group (75.0%, 64.0%), but there was no significant difference when compared with the COPD group (90.2%, 82.0%). Regarding comorbid disease, the proportion of connective tissue disease (CTD) in the CPFE group (10.4%) and the ILD group (13.4%) was higher than that in the COPD group (1.5%). The proportion of hyperlipidemia in the CPFE group (8.3%) was higher than that in the COPD group (1.5%) and the ILD group (1.2%). There were differences in the abnormal proportion of antinuclear antibody among the three groups, but no significant difference was found when compared with the CPFE group alone. The CPFE group (46.9%, 12.5%) and the ILD group (54.9%, 9.8%) were significantly higher than the COPD group (34.6%, 2.3%) in terms of carcinoembryonic antigen (CEA) abnormal proportion and cancer rate. In terms of the prevalence of pulmonary hypertension, the CPFE group (41.7%) > the COPD group (33.1%) > the ILD group (32.9%) was shown, but no statistical significance was found among the three groups.Conclusions Male and smoking are not only risk factors for COPD but also for CPFE. At the same time, the suffering of CPFE may be affected by immune factors and hyperlipidemia. The proportion of CPFE patients complicated with cancer and CEA abnormalities is higher than COPD patients. The severity of pulmonary hypertension in CPFE patients is significantly higher than the other two diseases.

Citation： WU Suji, WEI Yi, SHI Junqing, ZHANG Xu, XIE Min. Clinical characteristics and risk factors of combined pulmonary fibrosis and emphysema syndrome. Chinese Journal of Respiratory and Critical Care Medicine, 2021, 20(1): 4-11. doi: 10.7507/1671-6205.201909048 Copy

1.	Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognis ed entity. Eur Respir J, 2005, 26(4): 586-593.
2.	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.
3.	Grubstein A, Bendayan D, Schactman I, et al. Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the lite rature. Respir Med, 2005, 99(8): 948-954.
4.	Katzenstein AL. Republished: smoking-related interstitial fibrosis (SRIF): pathologic findings and distinction from other chronic fibrosing lung diseases. Postgrad Med J, 2014, 90(1068): 597-602.
5.	李松桃, 方明亮, 唐旭毛, 等. 国内肺纤维化合并肺气肿综合征临床特点的系统分析. 国际呼吸杂志, 2017, 37(2): 124-129.
6.	Sauleda J, Núñez B, Sala E, et al. Idiopathic pulmonary fibrosis: epidemiology, natural history, phenotypes. Med Sci (Basel), 2018, 6(4): 110.
7.	李锋, 徐蒙蒙, 王沐昀, 等. 建立小鼠肺纤维化合并肺气肿模型的实验研究. 中国呼吸与危重监护杂志, 2018, 17(6): 604-608.
8.	Cottin V, Nunes H, Mouthon L, et al. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis Rheum, 2011, 63(1): 295-304.
9.	Takizawa H, Tanaka M, Takami K, et al. Increased expression of transforming growth factor-β1 in small airway epithelium from tobacco smokers and patients with chronic obstructive pulmonary disease (COPD). Am J Respir Crit Care Med, 2001, 163(6): 1476-1483.
10.	Antoniou KM, Walsh SL, Hansell DM, et al. Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung. Respirology, 2013, 18(8): 1191-1196.
11.	Antoniou KM, Margaritopoulos GA, Goh NS, et al. Combined pulmonary fibrosis and emphysema in scleroderma-related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension. Arthritis Rheumatol, 2016, 68(4): 1004-1012.
12.	Jacob J, Song JW, Yoon HY, et al. Prevalence and effects of emphysema in never-smokers with rheumatoid arthritis interstitial lung disease. EBioMedicine, 2018, 28: 303-310.
13.	Champtiaux N, Cottin V, Chassagnon G, et al. Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality. Semin Arthritis Rheum, 2019, 49(1): 98-104.
14.	Nunes H, Monnet I, Kannengiesser C, et al. Is telomeropathy the explanation for combined pulmonary fibrosis and emphysema syndrome?: report of a family with TERT mutation. Am J Respir Crit Care Med, 2014, 189(6): 753-754.
15.	Casali L, Carratu P, Sofia M. Clinical variability of respiratory pulmonary hypertension: implications for diagnosis and management. Multidiscip Respir Med, 2013, 8(1): 72.
16.	Auriel E, Bornstein NM, Berenyi E, et al. Clinical, radiological and pathological correlates of leukoaraiosis. Acta Neurol Scand, 2011, 123(1): 41-47.
17.	Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Respir J, 2015, 46(4): 1855-1856.
18.	Oldham JM, Collard HR. Comorbid conditions in idiopathic pulmonary fibrosis: recognition and management. Front Med (Lausanne), 2017, 4: 123.
19.	Awano N, Inomata M, Ikushima S, et al. Histological analysis of vasculopathy associated with pulmonary hypertension in combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis or emphysema alone. Histopathology, 2017, 70(6): 896-905.
20.	Jacob J, Bartholmai BJ, Rajagopalan S, et al. Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema. Respirology, 2018, 23(6): 593-599.
21.	Oh JY, Lee YS, Min KH, et al. Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema: a retrospective study. Medicine (Baltimore), 2018, 97(31): e11683.
22.	Kwak N, Park CM, Lee J, et al. Lung cancer risk among patients with combined pulmonary fibrosis and emphysema. Respir Med, 2014, 108(3): 524-530.
23.	Zhang M, Yoshizawa A, Kawakami S, et al. The histological characteristics and clinical outcomes of lung cancer in patients with combined pulmonary fibrosis and emphysema. Cancer Med, 2016, 5(10): 2721-2730.
24.	Zhang LJ, Zhang CL, Dong FS, et al. Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis. BMC Pulm Med, 2016, 16(1): 137.
25.	Gao L, Xie S, Liu H, et al. Lung cancer in patients with combined pulmonary fibrosis and emphysema revisited with the 2015 World Health Organization classification of lung tumors. Clin Respir J, 2018, 12(2): 652-658.
26.	Mimae T, Suzuki K, Tsuboi M, et al. Surgical outcomes of lung cancer in patients with combined pulmonary fibrosis and emphysema. Ann Surg Oncol, 2015, 22(3): S1371-S1379.
27.	Lee T, Park JY, Lee HY, et al. Lung cancer in patients with idiopathic pulmonary fibrosis: clinical characteristics and impact on survival. Respir Med, 2014, 108(10): 1549-1555.
28.	Calio A, Lever V, Rossi A, et al. Increased frequency of bronchiolar histotypes in lung carcinomas associated with idiopathic pulmonary fibrosis. Histopathology, 2017, 71(5): 725-735.
29.	Oh SY, Kim MY, Kim JE, et al. Evolving early lung cancers detected during follow-up of idiopathic interstitial pneumonia: serial CT features. Am J Roentgenol, 2015, 204(6): 1190-1196.
30.	Lee BW, Wain JC, Kelsey KT, et al. Association of cigarette smoking and asbestos exposure with location and histology of lung cancer. Am J Respir Crit Care Med, 1998, 157(3 Pt 1): 748-755.
31.	Maeda R, Funasaki A, Motono N, et al. Combined pulmonary fibrosis and emphysema predicts recurrence following surgery in patients with stage Ⅰ non-small cell lung cancer. Med Oncol, 2018, 35(3): 31.
32.	Takenaka T, Furuya K, Yamazaki K, et al. The prognostic impact of combined pulmonary fibrosis and emphysema in patients with clinical stage ⅠA non-small cell lung cancer. Surg Today, 2018, 48(2): 229-235.

1. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognis ed entity. Eur Respir J, 2005, 26(4): 586-593.
2. 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.
3. Grubstein A, Bendayan D, Schactman I, et al. Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the lite rature. Respir Med, 2005, 99(8): 948-954.
4. Katzenstein AL. Republished: smoking-related interstitial fibrosis (SRIF): pathologic findings and distinction from other chronic fibrosing lung diseases. Postgrad Med J, 2014, 90(1068): 597-602.
5. 李松桃, 方明亮, 唐旭毛, 等. 国内肺纤维化合并肺气肿综合征临床特点的系统分析. 国际呼吸杂志, 2017, 37(2): 124-129.
6. Sauleda J, Núñez B, Sala E, et al. Idiopathic pulmonary fibrosis: epidemiology, natural history, phenotypes. Med Sci (Basel), 2018, 6(4): 110.
7. 李锋, 徐蒙蒙, 王沐昀, 等. 建立小鼠肺纤维化合并肺气肿模型的实验研究. 中国呼吸与危重监护杂志, 2018, 17(6): 604-608.
8. Cottin V, Nunes H, Mouthon L, et al. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis Rheum, 2011, 63(1): 295-304.
9. Takizawa H, Tanaka M, Takami K, et al. Increased expression of transforming growth factor-β1 in small airway epithelium from tobacco smokers and patients with chronic obstructive pulmonary disease (COPD). Am J Respir Crit Care Med, 2001, 163(6): 1476-1483.
10. Antoniou KM, Walsh SL, Hansell DM, et al. Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung. Respirology, 2013, 18(8): 1191-1196.
11. Antoniou KM, Margaritopoulos GA, Goh NS, et al. Combined pulmonary fibrosis and emphysema in scleroderma-related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension. Arthritis Rheumatol, 2016, 68(4): 1004-1012.
12. Jacob J, Song JW, Yoon HY, et al. Prevalence and effects of emphysema in never-smokers with rheumatoid arthritis interstitial lung disease. EBioMedicine, 2018, 28: 303-310.
13. Champtiaux N, Cottin V, Chassagnon G, et al. Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality. Semin Arthritis Rheum, 2019, 49(1): 98-104.
14. Nunes H, Monnet I, Kannengiesser C, et al. Is telomeropathy the explanation for combined pulmonary fibrosis and emphysema syndrome?: report of a family with TERT mutation. Am J Respir Crit Care Med, 2014, 189(6): 753-754.
15. Casali L, Carratu P, Sofia M. Clinical variability of respiratory pulmonary hypertension: implications for diagnosis and management. Multidiscip Respir Med, 2013, 8(1): 72.
16. Auriel E, Bornstein NM, Berenyi E, et al. Clinical, radiological and pathological correlates of leukoaraiosis. Acta Neurol Scand, 2011, 123(1): 41-47.
17. Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Respir J, 2015, 46(4): 1855-1856.
18. Oldham JM, Collard HR. Comorbid conditions in idiopathic pulmonary fibrosis: recognition and management. Front Med (Lausanne), 2017, 4: 123.
19. Awano N, Inomata M, Ikushima S, et al. Histological analysis of vasculopathy associated with pulmonary hypertension in combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis or emphysema alone. Histopathology, 2017, 70(6): 896-905.
20. Jacob J, Bartholmai BJ, Rajagopalan S, et al. Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema. Respirology, 2018, 23(6): 593-599.
21. Oh JY, Lee YS, Min KH, et al. Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema: a retrospective study. Medicine (Baltimore), 2018, 97(31): e11683.
22. Kwak N, Park CM, Lee J, et al. Lung cancer risk among patients with combined pulmonary fibrosis and emphysema. Respir Med, 2014, 108(3): 524-530.
23. Zhang M, Yoshizawa A, Kawakami S, et al. The histological characteristics and clinical outcomes of lung cancer in patients with combined pulmonary fibrosis and emphysema. Cancer Med, 2016, 5(10): 2721-2730.
24. Zhang LJ, Zhang CL, Dong FS, et al. Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis. BMC Pulm Med, 2016, 16(1): 137.
25. Gao L, Xie S, Liu H, et al. Lung cancer in patients with combined pulmonary fibrosis and emphysema revisited with the 2015 World Health Organization classification of lung tumors. Clin Respir J, 2018, 12(2): 652-658.
26. Mimae T, Suzuki K, Tsuboi M, et al. Surgical outcomes of lung cancer in patients with combined pulmonary fibrosis and emphysema. Ann Surg Oncol, 2015, 22(3): S1371-S1379.
27. Lee T, Park JY, Lee HY, et al. Lung cancer in patients with idiopathic pulmonary fibrosis: clinical characteristics and impact on survival. Respir Med, 2014, 108(10): 1549-1555.
28. Calio A, Lever V, Rossi A, et al. Increased frequency of bronchiolar histotypes in lung carcinomas associated with idiopathic pulmonary fibrosis. Histopathology, 2017, 71(5): 725-735.
29. Oh SY, Kim MY, Kim JE, et al. Evolving early lung cancers detected during follow-up of idiopathic interstitial pneumonia: serial CT features. Am J Roentgenol, 2015, 204(6): 1190-1196.
30. Lee BW, Wain JC, Kelsey KT, et al. Association of cigarette smoking and asbestos exposure with location and histology of lung cancer. Am J Respir Crit Care Med, 1998, 157(3 Pt 1): 748-755.
31. Maeda R, Funasaki A, Motono N, et al. Combined pulmonary fibrosis and emphysema predicts recurrence following surgery in patients with stage Ⅰ non-small cell lung cancer. Med Oncol, 2018, 35(3): 31.
32. Takenaka T, Furuya K, Yamazaki K, et al. The prognostic impact of combined pulmonary fibrosis and emphysema in patients with clinical stage ⅠA non-small cell lung cancer. Surg Today, 2018, 48(2): 229-235.

Chinese Journal of Respiratory and Critical Care Medicine

Clinical characteristics and risk factors of combined pulmonary fibrosis and emphysema syndrome

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