Analysis of clinical characteristics of combined pulmonary fibrosis emphysema syndrome in 20 patients with connective tissue disease_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

WANG Lan ¹ , CHAI Hua ^2,3 , LIANG Binmiao ¹ , ZHANG Rui ⁴ , LU Chunyan ⁵ , LV Xiafei ⁵ , YI Qun ¹ ,  OU Xuemei ¹

1. Department of Respiratory and Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Academic Affairs, West China School of Medicine, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
3. Laboratory of Cardiovascular Diseases, Regenerative Medicine Research Center, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P. R. China;
4. Information Center, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P. R. China;
5. Department of Radiology, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

OU Xuemei, Email: ouxuemei@163.com

Keywords：

Combined pulmonary fibrosis and emphysema syndrome; Connective tissue disease

DOI：

10.7507/1671-6205.201705005

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Objective To improve the knowledge and diagnostic accuracy of combined pulmonary fibrosis and emphysema (CPFE) syndrome in connective tissue diseases (CTD) by summarizing the clinical characteristics of 20 CTD patients with CPFE and reviewing literatures. Methods The medical records of 20 CTD patients with CPFE from January 2011 to June 2015 were retrospectively analyzed. Results There were 11 males and 9 females. The average age was 47 years. Among them, 4 patients were smokers and 15 patients were nonsmokers. The average duration of CTD was 3.5 years with an average onset age of 41 years. Respiratory symptoms were reported in 17 patients and Velcro rale was found in 9 patients; The most common type of CTD disease in these 20 patients was inflammatory myopathy (9 patients, 45%) followed by systemic sclerosis (SSc) (4 patients, 20%). High resolution computerized tomography of lung showed typical radiological features of CPFE containing fibrosis lesions predominantly distributed in the subpleural (14 patients) and basal (18 patients) parts and emphysema mainly located in upper zones. Relatively normal results of lung volume and ventilation function, and markedly reduced carbon monoxide transfer capacity were observed. One patient was confirmed with pulmonary hypertension and 1 patient died from severe inflammation and acute respiratory distress syndrome. Conclusions The CPFE syndrome can be identified in CTD patients as an entity with male predominance, especially among patients with inflammatory myopathy and SSc. Higher risk of secondary pulmonary hypertension and acute lung injury in these patients may increase mortality. Early differentiation of CPFE from pure interstitial lung disease in CTD patients could be helpful in improving prognosis.

Citation： WANG Lan, CHAI Hua, LIANG Binmiao, ZHANG Rui, LU Chunyan, LV Xiafei, YI Qun, OU Xuemei. Analysis of clinical characteristics of combined pulmonary fibrosis emphysema syndrome in 20 patients with connective tissue disease. Chinese Journal of Respiratory and Critical Care Medicine, 2017, 16(5): 484-489. doi: 10.7507/1671-6205.201705005 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.	Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012, 141(1): 222-231.
3.	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.
4.	Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum, 1988, 31(3): 315-324.
5.	Troyanov Y, Targoff IN, Tremblay JL, et al. Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine 2005, 84(4): 231-249.
6.	Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Annals of the rheumatic diseases 2002, 61(6): 554-558.
7.	Alarcon-Segovia D, Cardiel MH. Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. The Journal of rheumatology 1989, 16(3): 328-334.
8.	Cottin V, Cordier JF. Combined pulmonary fibrosis and emphysema in connective tissue disease. Current opinion in pulmonary medicine 2012, 18(5): 418-427.
9.	Cottin V, Nunes H, Mouthon L, et al. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis and rheumatism 2011, 63(1): 295-304.
10.	Fairweather D, Frisancho-Kiss S, Rose NR. Sex Differences in Autoimmune Disease from a Pathological Perspective. The American Journal of Pathology 2008, 173(3): 600-609.
11.	Cottin V. The impact of emphysema in pulmonary fibrosis. European respiratory review : an official journal of the European Respiratory Society 2013, 22(128): 153-157.
12.	Smith BM, Austin JH, Newell JD, Jr., et al. Pulmonary emphysema subtypes on computed tomography: the MESA COPD study. The American journal of medicine 2014, 127(1): 94 e97-23.
13.	Kitaguchi Y, Fujimoto K, Hanaoka M, et al. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology (Carlton, Vic) 2010, 15(2): 265-271.
14.	Nair A, Hansell DM. High-resolution computed tomography features of smoking-related interstitial lung disease. Seminars in ultrasound, CT, and MR 2014, 35(1): 59-71.
15.	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.
16.	Kitaguchi Y, Fujimoto K, Hayashi R, et al. Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up. Respiratory medicine 2013, 107(12): 1986-1992.
17.	Washko GR, Hunninghake GM, Fernandez IE, et al. Lung Volumes and Emphysema in Smokers with Interstitial Lung Abnormalities. New England Journal of Medicine 2011, 364(10): 897-906.
18.	Akagi T, Matsumoto T, Harada T, et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respiratory medicine 2009, 103(8): 1209-1215.
19.	Cottin V, Le Pavec J, Prevot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. The European respiratory journal 2010, 35(1): 105-111.
20.	Kurashima K, Takayanagi N, Tsuchiya N, et al. The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis. Respirology (Carlton, Vic) 2010, 15(5): 843-848.
21.	Mejia M, Carrillo G, Rojas-Serrano J, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009, 136(1): 10-15.
22.	Jankowich MD, Rounds S. Combined pulmonary fibrosis and emphysema alters physiology but has similar mortality to pulmonary fibrosis without emphysema. Lung 2010, 188(5): 365-373.

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. Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012, 141(1): 222-231.
3. 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.
4. Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum, 1988, 31(3): 315-324.
5. Troyanov Y, Targoff IN, Tremblay JL, et al. Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine 2005, 84(4): 231-249.
6. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Annals of the rheumatic diseases 2002, 61(6): 554-558.
7. Alarcon-Segovia D, Cardiel MH. Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. The Journal of rheumatology 1989, 16(3): 328-334.
8. Cottin V, Cordier JF. Combined pulmonary fibrosis and emphysema in connective tissue disease. Current opinion in pulmonary medicine 2012, 18(5): 418-427.
9. Cottin V, Nunes H, Mouthon L, et al. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis and rheumatism 2011, 63(1): 295-304.
10. Fairweather D, Frisancho-Kiss S, Rose NR. Sex Differences in Autoimmune Disease from a Pathological Perspective. The American Journal of Pathology 2008, 173(3): 600-609.
11. Cottin V. The impact of emphysema in pulmonary fibrosis. European respiratory review : an official journal of the European Respiratory Society 2013, 22(128): 153-157.
12. Smith BM, Austin JH, Newell JD, Jr., et al. Pulmonary emphysema subtypes on computed tomography: the MESA COPD study. The American journal of medicine 2014, 127(1): 94 e97-23.
13. Kitaguchi Y, Fujimoto K, Hanaoka M, et al. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology (Carlton, Vic) 2010, 15(2): 265-271.
14. Nair A, Hansell DM. High-resolution computed tomography features of smoking-related interstitial lung disease. Seminars in ultrasound, CT, and MR 2014, 35(1): 59-71.
15. 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.
16. Kitaguchi Y, Fujimoto K, Hayashi R, et al. Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up. Respiratory medicine 2013, 107(12): 1986-1992.
17. Washko GR, Hunninghake GM, Fernandez IE, et al. Lung Volumes and Emphysema in Smokers with Interstitial Lung Abnormalities. New England Journal of Medicine 2011, 364(10): 897-906.
18. Akagi T, Matsumoto T, Harada T, et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respiratory medicine 2009, 103(8): 1209-1215.
19. Cottin V, Le Pavec J, Prevot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. The European respiratory journal 2010, 35(1): 105-111.
20. Kurashima K, Takayanagi N, Tsuchiya N, et al. The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis. Respirology (Carlton, Vic) 2010, 15(5): 843-848.
21. Mejia M, Carrillo G, Rojas-Serrano J, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009, 136(1): 10-15.
22. Jankowich MD, Rounds S. Combined pulmonary fibrosis and emphysema alters physiology but has similar mortality to pulmonary fibrosis without emphysema. Lung 2010, 188(5): 365-373.

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