Comparison of the clinical features of combined pulmonary fibrosis and emphysema and idiopathic pulmonary fibrosis_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

LIN Minjie ¹ , ZHANG Yingwei ² , CHENG Lulu ² , LIU Xiaoqing ² , WANG Yanhong ¹ ,  QIU Yuying ¹

1. Department of Respiratory and Critical Care Medicine,Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, Nanjing, Jiangsu 210008, P. R. China;
2. Department of Respiratory and Critical Care Medicine,Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, Jiangsu 210008, P. R. China;

Corresponding author：

QIU Yuying, Email: qiuyuying1402@sina.com

Keywords：

Combined pulmonary fibrosis and emphysema; Idiopathic pulmonary fibrosis; Clinical manifestation

DOI：

10.7507/1671-6205.201911017

Video：

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Objectives To compare the clinical features of combined pulmonary fibrosis and emphysema (CPFE) and idiopathic pulmonary fibrosis (IPF).Methods Eighty-three patients diagnosed as CPFE or IPF for the first time were retrospectively analyzed from June 2014 to July 2018 in Nanjing Drum Tower Hospital, including 47 patients in the CPFE group and 36 in the IPF group. The demographic characteristics, clinical manifestations, pulmonary function, cardiac ultrasound, blood gas analysis and prognosis of the two groups were compared.Results The proportion of smokers in the CPFE group was higher than IPF group (P<0.05), but dyspnea was lower (P<0.05). The FVC, FVC%pred, FEV₁, FEV₁%pred and VC% of the CPFE group were higher than IPF group (P<0.05), while FEV₁/FVC%pred in the IPF group was higher than CPFE group (P<0.05). D_LCO/VA%pred of CPFE group decreased more significantly than IPF group (P<0.05), RV/TLC%pred of CPFE group increased annually, while decreased annually in IPF group (P<0.01). The RV%pred of CPFE increased annually, while that of IPF group decreased annually (P<0.05). There was no significant difference in arterial oxygen pressure and pulmonary artery pressure between the two groups. As for prognosis, the 1- and 3-year survival rate of the CPFE group were 87.9% and 73.8% respectively, those of the IPF group were 84.1% and 65.8% respectively, and no significantly difference was observed between two groups (P=0.95).Conclusions Compared with IPF, patients with CPFE usually have more smokers, less proportion of dyspnea, almost normal lung volume, more rapidly decreased D_LCO/VA%pred, and no significant difference in prognosis.

Citation： LIN Minjie, ZHANG Yingwei, CHENG Lulu, LIU Xiaoqing, WANG Yanhong, QIU Yuying. Comparison of the clinical features of combined pulmonary fibrosis and emphysema and idiopathic pulmonary fibrosis. Chinese Journal of Respiratory and Critical Care Medicine, 2020, 19(6): 548-553. doi: 10.7507/1671-6205.201911017 Copy

1.	Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J, 2005, 26(4): 586-593.
2.	Suzuki A, Kondoh Y. The clinical impact of major comorbidities on idiopathic pulmonary fibrosis. Respir Investig, 2017, 55(2): 94-103.
3.	Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med, 2018, 378(19): 1811-1823.
4.	黄海, 郑臻, 陈依林, 等. 特发性肺纤维化合并肺气肿与未合并肺气肿患者的临床差异. 国际呼吸杂志, 2014, 34(4): 254-256.
5.	Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med, 2011, 183(6): 788-824.
6.	Sugino K, Ishida F, Kikuchi N, et al. Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone. Respirology, 2014, 19(2): 239-245.
7.	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.
8.	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.
9.	Pepin EW, Verma N, Mehta HJ, et al. Combined pulmonary fibrosis and emphysema. Ann Am Thorac Soc, 2018, 15(1): 110-112.
10.	Morse D, Rosas IO. Tobacco smoke-induced lung fibrosis and emphysema. Annu Rev Physiol, 2014, 76: 493-513.
11.	Ryu JH, Colby TV, Hartman TE, et al. Smoking-related interstitial lung diseases: a concise review. Eur Respir J, 2001, 17(1): 122-132.
12.	Papaioannou AI, Kostikas K, Manali ED, et al. Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract. Respir Med, 2016, 117: 14-26.
13.	Malli F, Papakosta D, Antoniou K, et al. Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort. ERJ Open Res, 2019, 5(1): 00014-2018.
14.	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.
15.	Çiftci F, Gülpınar B, Atasoy Ç, et al. Combined pulmonary fibrosis and emphysema: how does cohabitation affect respiratory functions?. Adv Med Sci, 2019, 64(2): 285-291.
16.	Pecchio F, Rapellino M, Ricci E, et al. TA 4-SCC versus CEA sensitivity for lung cancer. Tumori, 1988, 74(4): 393-395.
17.	Kulpa J, Wojcik E, Reinfuss M, et al. Carcinoembryonic antigen, squamous cell carcinoma antigen, CYFRA 21-1, and neuron-specific enolase in squamous cell lung cancer patients. Clin Chem, 2002, 48(11): 1931-1937.
18.	Brechot JM, Chevret S, Nataf J, et al. Diagnostic and prognostic value of Cyfra 21-1 compared with other tumour markers in patients with non-small cell lung cancer: a prospective study of 116 patients. Eur J Cancer, 1997, 33(3): 385-391.
19.	黄妩姣, 吴子安, 易四维, 等. 比较不同方法检测血清 CYFRA21-1 对肺癌的临床诊断价值. 广州医学院学报, 2010, 38(1): 44-47.
20.	Kumar A, Cherian SV, Vassallo R, et al. Current concepts in pathogenesis, diagnosis, and management of smoking-related interstitial lung diseases. Chest, 2018, 154(2): 394-408.
21.	Kohashi Y, Arai T, Sugimoto C, et al. Clinical impact of emphysema evaluated by high-resolution computed tomography on idiopathic pulmonary fibrosis diagnosed by surgical lung biopsy. Respiration, 2016, 92(4): 220-228.
22.	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.

1. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J, 2005, 26(4): 586-593.
2. Suzuki A, Kondoh Y. The clinical impact of major comorbidities on idiopathic pulmonary fibrosis. Respir Investig, 2017, 55(2): 94-103.
3. Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med, 2018, 378(19): 1811-1823.
4. 黄海, 郑臻, 陈依林, 等. 特发性肺纤维化合并肺气肿与未合并肺气肿患者的临床差异. 国际呼吸杂志, 2014, 34(4): 254-256.
5. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med, 2011, 183(6): 788-824.
6. Sugino K, Ishida F, Kikuchi N, et al. Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone. Respirology, 2014, 19(2): 239-245.
7. 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.
8. 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.
9. Pepin EW, Verma N, Mehta HJ, et al. Combined pulmonary fibrosis and emphysema. Ann Am Thorac Soc, 2018, 15(1): 110-112.
10. Morse D, Rosas IO. Tobacco smoke-induced lung fibrosis and emphysema. Annu Rev Physiol, 2014, 76: 493-513.
11. Ryu JH, Colby TV, Hartman TE, et al. Smoking-related interstitial lung diseases: a concise review. Eur Respir J, 2001, 17(1): 122-132.
12. Papaioannou AI, Kostikas K, Manali ED, et al. Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract. Respir Med, 2016, 117: 14-26.
13. Malli F, Papakosta D, Antoniou K, et al. Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort. ERJ Open Res, 2019, 5(1): 00014-2018.
14. 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.
15. Çiftci F, Gülpınar B, Atasoy Ç, et al. Combined pulmonary fibrosis and emphysema: how does cohabitation affect respiratory functions?. Adv Med Sci, 2019, 64(2): 285-291.
16. Pecchio F, Rapellino M, Ricci E, et al. TA 4-SCC versus CEA sensitivity for lung cancer. Tumori, 1988, 74(4): 393-395.
17. Kulpa J, Wojcik E, Reinfuss M, et al. Carcinoembryonic antigen, squamous cell carcinoma antigen, CYFRA 21-1, and neuron-specific enolase in squamous cell lung cancer patients. Clin Chem, 2002, 48(11): 1931-1937.
18. Brechot JM, Chevret S, Nataf J, et al. Diagnostic and prognostic value of Cyfra 21-1 compared with other tumour markers in patients with non-small cell lung cancer: a prospective study of 116 patients. Eur J Cancer, 1997, 33(3): 385-391.
19. 黄妩姣, 吴子安, 易四维, 等. 比较不同方法检测血清 CYFRA21-1 对肺癌的临床诊断价值. 广州医学院学报, 2010, 38(1): 44-47.
20. Kumar A, Cherian SV, Vassallo R, et al. Current concepts in pathogenesis, diagnosis, and management of smoking-related interstitial lung diseases. Chest, 2018, 154(2): 394-408.
21. Kohashi Y, Arai T, Sugimoto C, et al. Clinical impact of emphysema evaluated by high-resolution computed tomography on idiopathic pulmonary fibrosis diagnosed by surgical lung biopsy. Respiration, 2016, 92(4): 220-228.
22. 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.

Chinese Journal of Respiratory and Critical Care Medicine

Comparison of the clinical features of combined pulmonary fibrosis and emphysema and idiopathic pulmonary fibrosis

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