Combined pulmonary fibrosis and emphysema syndrome: clinical, functional, and radiological assessment
Egyptian Journal of Bronchology volume 12, pages 76–82 (2018)
The coexistence of pulmonary fibrosis and emphysema is increasingly recognized.
To assess the clinical, physiological and radiological characteristics of patients with combined pulmonary fibrosis and emphysema (CPFE) and compare it with patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) alone.
Patients and methods
One hundred-twenty patients were enrolled and divided into three groups; 40 had COPD based on poorly reversible airflow obstruction in spirometry; 40 had ILD based on high-resolution computed tomography (HRCT); and 40 had CPFE based on the presence of emphysematous changes in the upper lung zones and pulmonary fibrosis in lower zones in HRCT. Modified Medical Research Council dyspnea scale, arterial blood gas analysis, spirometry, diffusion capacity for carbon monoxide (DLCO), polythesmography, HRCT chest, and echocardiography were done.
More than 57% of patients with CPFE were men and the majority of them were smokers. There was no significant difference in dyspnea grade between CPFE group and other groups (P>0.05). The rate of exacerbation per year was significantly higher in the CPFE group (4.2±1.02) compared with either COPD group (3.33±1.56) or ILD group (3.15 ±1.05). CPFE patients had both emphysematous and fibrotic changes on radiological examination. Lung volumes were preserved but DLCO% was significantly lower and the mean pulmonary artery systolic pressure was significantly higher in the CPFE group compared with COPD and ILD.
CPFE is a distinct syndrome that has characteristic radiological findings and lung function profile with a significant reduction of DLCO and a significant increase in pulmonary artery systolic pressure.
Auerbach O, Garfinkel L, Hammond EC. Relation of smoking and age to findings in lung parenchyma: a microscopic study. Chest 1974; 65:29–35.
Cottin V, Nunes H, Brillet PY, Delaval P, Delaval P, Devouassoux G, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26:586–593.
Ryerson CJ, Hartman T, Elicker BM, Ley B, Lee JS, Abbritti M, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest 2013; 144:234–240.
Sakai F, Tominaga J, Kaga A, Usui Y, Kanazawa M, Ogura T, et al. Imaging diagnosisof interstitial pneumonia with emphysema (combinedpulmonary fibrosis and emphysema). Pulm Med 2012; 2012:816541.
Fujimoto K, Kitaguchi Y, Kubo K, Honda T. Clinical analysis of chronic obstructive pulmonary disease phenotypes classified using high-resolution computed tomography. Respirology 2006; 11:731–740.
Cottin V, Nunes H, Mouthon L, Gamondes D, Lazor R, Hachulla E. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis Rheum 2011; 63:295–304.
Jankowich DM, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012; 14:222–231.
Jankowich MD, Polsky M, Klein M, Rounds S. Heterogeneity in combined pulmonary fibrosis and emphysema. Respiration 2008; 75:411–417.
Global Initiative for Chronic Obstructive Lung Disease (GOLD): Global Strategy for the Diagnosis, Management, and Prevention of COPD. 2017 Global Initiative for Chronic Obstructive Lung Disease, Inc.; GOLD Website, www.goldcopd.org page 4.
Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. ATS/ERS/JRS/ALAT committee on idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183:788–824.
Bestall JC, Paul EA, Garrod R, Garnham R, Jones PW, Wedzicha JA. Usefulness of the Medical Research Council (MRC) dyspnoea scale as a measure of disability in patients with chronic obstructive pulmonary disease. Thorax 1999; 54:581–586.
Weiner P, Novitzky T, Weiner D, Beckerman M. Chronic obstructive pulmonary disorder (COPD) patients with the syndrome of combined pulmonary fibrosis and emphysema, compared to patients with emphysema alone. Harefuah 2013; 152:294–298 307, 308
Akagi T, Matsumoto T, Harada T, Tanaka M, Kuraki T, Fujita M, et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med 2009; 103:1209–1215.
Sugino K, Ishida F, Kikuchi N, Hirota N, Sano G, Sato K, et al. Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone. Respirology 2014; 19:239–245.
Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J RespirCrit Care Med 2006; 174:810–816.
Tomioka H, Mamesaya N, Yamashita S, Kida Y, Kaneko M, Sakai H. Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease. BMJ Open Respir Res 2016; 3:96–99.
Jankowich MD, Rounds S. Combined pulmonary fibrosisand emphysema alters physiology but has similar mortality topulmonary fibrosis without emphysema. Lung 2010; 188:365–370.
Kitaguchi Y, Fujimoto K, Hanaoka M, Kawakami S, Honda T, Kubo K. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology 2010; 15:265–271.
Mejia M, Carrillo G, Rojas-Serrano J, Estrada A, Suarez T, Alonso D, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associatedwith severe pulmonary arterial hypertension. Chest 2009; 136:10–15.
Caminati A, Cassandro R, Harari S. Pulmonaryhypertension in chronic interstitial lung diseases. Eur Respir Rev 2013; 22:292–301.
Cottin V, Le Pavec J, Prévot G, Mal H, Humbert M, Simonneau G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosisand emphysema syndrome. Eur Respir J 2010; 35:105–111.
Washko GR, Hunninghake GM, Fernandez IE, Nishino M, Okajima Y, Yamashiro T, et al. Lung volumes and emphysema in smokers with interstitial lung abnormalities. N Engl J Med 2011; 364:897–906.
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Ghanem, M.K., Makhlouf, H.A., Hassan, A.A. et al. Combined pulmonary fibrosis and emphysema syndrome: clinical, functional, and radiological assessment. Egypt J Bronchol 12, 76–82 (2018). https://doi.org/10.4103/ejb.ejb_51_17
- diffusion capacity for carbon monoxide
- high-resolution computed tomography
- pulmonary hypertension