Combined pulmonary fibrosis and emphysema (CPFE): An entity different from emphysema or pulmonary fibrosis alone

Journal of Thoracic Disease

Volumn 7, Issue 4, 2015, Pages 767-779

  Lin, Huijin ^a   Jiang, Shanping ^a  

^a SUN YAT SEN UNIVERSITY   (China)

Author keywords

Chronic obstructive pulmonary disease (COPD); Idiopathic interstitial pneumonia (IIP); Idiopathic pulmonary fibrosis (IPF); Pulmonary emphysema; Pulmonary hypertension

Indexed keywords

ACETYLCYSTEINE; AZATHIOPRINE; BETA 2 ADRENERGIC RECEPTOR STIMULATING AGENT; BRONCHODILATING AGENT; CLUB CELL SECRETORY PROTEIN 16; CORTICOSTEROID; ENDOTHELIN RECEPTOR ANTAGONIST; INFLUENZA VACCINE; MUSCARINIC RECEPTOR BLOCKING AGENT; NINTEDANIB; OXYGEN; PHOSPHODIESTERASE V INHIBITOR; PIRFENIDONE; PLACEBO; PNEUMOCOCCUS VACCINE; PREDNISOLONE; PROSTANOID; SECRETORY PROTEIN; SURFACTANT PROTEIN C; UNCLASSIFIED DRUG;

ACUTE LUNG INJURY; ARTERIAL OXYGEN TENSION; ARTICLE; BLOOD GAS ANALYSIS; CLINICAL PRACTICE; COMBINED LUNG FIBROSIS AND EMPHYSEMA; CONNECTIVE TISSUE DISEASE; CONTROLLED CLINICAL TRIAL (TOPIC); DIFFERENTIAL DIAGNOSIS; DISEASE SEVERITY; DYSPNEA; ECHOGRAPHY; EMPHYSEMA; GENE MUTATION; GENETIC SUSCEPTIBILITY; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HUMAN; HYPOXEMIA; INFLUENZA; LUNG ALVEOLUS OXYGEN TENSION; LUNG CANCER; LUNG FIBROSIS; LUNG FUNCTION TEST; LUNG GAS EXCHANGE; LUNG TRANSPLANTATION; LUNG VOLUME; OCCUPATIONAL EXPOSURE; PATHOGENESIS; PATIENT CARE; PHASE 3 CLINICAL TRIAL (TOPIC); PROGNOSIS; PULMONARY HYPERTENSION; SMOKING; SMOKING CESSATION; STREPTOCOCCUS PNEUMONIA;

EID: 84928567599     PISSN: 20721439     EISSN: 20776624     Source Type: Journal    
DOI: 10.3978/j.issn.2072-1439.2015.04.17     Document Type: Article

References (69)

1. Snider GL, Kleinerman J, Thurlbeck WM, et al. The definition of emphysema. Report of a National Heart, Lung, and Blood Institute, Division of Lung Diseases workshop. Am Rev Respir Dis 1985;132:182-5.
2. Wiggins J, Strickland B, Turner-Warwick M. Combined cryptogenic fibrosing alveolitis and emphysema: the value of high resolution computed tomography in assessment. Respir Med 1990;84:365-9.
3. Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause. Respiration 1993;60:354-8.
4. Washko GR, Hunninghake GM, Fernandez IE, et al. Lung volumes and emphysema in smokers with interstitial lung abnormalities. N Engl J Med 2011;364:897-906.
5. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005;26:586-93.
6. Cottin V, Le Pavec J, Prévot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J 2010;35:105-11.
7. Kiakouama L, Cottin V, Glerant JC, et al. Conditions associated with severe carbon monoxide diffusion coefficient reduction. Respir Med 2011;105:1248-56.
8. 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 literature. Respir Med 2005;99:948-54.
9. Katzenstein AL. Republished: smoking-related interstitial fibrosis (SRIF): pathologic findings and distinction from other chronic fibrosing lung diseases. Postgrad Med J 2014;90:597-602.
10. Katzenstein AL, Mukhopadhyay S, Zanardi C, et al. Clinically occult interstitial fibrosis in smokers: classification and significance of a surprisingly common finding in lobectomy specimens. Hum Pathol 2010;41:316-25.
11. 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;183:788-824.
12. Nair A, Hansell DM. High-resolution computed tomography features of smoking-related interstitial lung disease. Semin Ultrasound CT MR 2014;35:59-71.
13. Papiris SA, Triantafillidou C, Manali ED, et al. Combined pulmonary fibrosis and emphysema. Expert Rev Respir Med 2013;7:19-31; quiz 32.
14. Ye Q, Huang K, Ding Y, et al. Cigarette smoking contributes to idiopathic pulmonary fibrosis associated with emphysema. Chin Med J (Engl) 2014;127:469-74.
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;144:234-40.
16. Mori K, Shirai T, Mikamo M, et al. Respiratory mechanics measured by forced oscillation technique in combined pulmonary fibrosis and emphysema. Respir Physiol Neurobiol 2013;185:235-40.
17. Sakai F, Tominaga J, Kaga A, et al. Imaging diagnosis of interstitial pneumonia with emphysema (combined pulmonary fibrosis and emphysema). Pulm Med 2012;2012:816541.
18. Fujimoto K, Kitaguchi Y, Kubo K, et al. Clinical analysis of chronic obstructive pulmonary disease phenotypes classified using high-resolution computed tomography. Respirology 2006;11:731-40.
19. Inomata M, Ikushima S, Awano N, et al. An autopsy study of combined pulmonary fibrosis and emphysema: correlations among clinical, radiological, and pathological features. BMC Pulm Med 2014;14:104.
20. 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;19:239-45.
21. Kim YJ, Shin SH, Park JW, et al. Annual Change in Pulmonary Function and Clinical Characteristics of Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis: Over a 3-Year Follow-up. Tuberc Respir Dis (Seoul) 2014;77:18-23.
22. 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. Respir Med 2013;107:1986-92.
23. Antoniou KM, Walsh SL, Hansell DM, et al. Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung. Respirology 2013;18:1191-6.
24. Camp PG, Coxson HO, Levy RD, et al. Sex differences in emphysema and airway disease in smokers. Chest 2009;136:1480-8.
25. Morse D, Rosas IO. Tobacco smoke-induced lung fibrosis and emphysema. Annu Rev Physiol 2014;76:493-513.
26. Lucattelli M, Bartalesi B, Cavarra E, et al. Is neutrophil elastase the missing link between emphysema and fibrosis? Evidence from two mouse models. Respir Res 2005;6:83.
27. Kitaguchi Y, Fujimoto K, Hanaoka M, et al. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology 2010;15:265-71.
28. Karkhanis VS, Joshi JM. Combined pulmonary fibrosis and emphysema in a tyre industry worker. Lung India 2012;29:273-6.
29. Roshan R, Guptal M, Kulshrestha R, et al. Combined pulmonary fibrosis and emphysema in a welder. Monaldi Arch Chest Dis 2012;77:26-8.
30. Tzouvelekis A, Zacharis G, Oikonomou A, et al. Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema. BMC Pulm Med 2013;13:31.
31. Hanaoka M, Ito M, Droma Y, Ushiki A, et al. Comparison of gene expression profiling between lung fibrotic and emphysematous tissues sampled from patients with combined pulmonary fibrosis and emphysema. Fibrogenesis Tissue Repair 2012;5:17.
32. Cottin V, Reix P, Khouatra C, et al. Combined pulmonary fibrosis and emphysema syndrome associated with familial SFTPC mutation. Thorax 2011;66:918-9.
33. Epaud R, Delestrain C, Louha M, et al. Combined pulmonary fibrosis and emphysema syndrome associated with ABCA3 mutations. Eur Respir J 2014;43:638-41.
34. Armanios M. Telomerase and idiopathic pulmonary fibrosis. Mutat Res 2012;730:52-8.
35. Armanios MY, Chen JJ, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med 2007;356:1317-26.
36. Alder JK, Guo N, Kembou F, et al. Telomere length is a determinant of emphysema susceptibility. Am J Respir Crit Care Med 2011;184:904-12.
37. 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;189:753-4.
38. Tasaka S, Mizoguchi K, Funatsu Y, et al. Cytokine profile of bronchoalveolar lavage fluid in patients with combined pulmonary fibrosis and emphysema. Respirology 2012;17:814-20.
39. Zhao Y, Cui A, Wang F, et al. Characteristics of pulmonary inflammation in combined pulmonary fibrosis and emphysema. Chin Med J (Engl) 2012;125:3015-21.
40. Price DB, Baker CL, Zou KH, et al. Real-world characterization and differentiation of the Global Initiative for Chronic Obstructive Lung Disease strategy classification. Int J Chron Obstruct Pulmon Dis 2014;9:551-61.
41. Lee AS, Mira-Avendano I, Ryu JH, et al. The burden of idiopathic pulmonary fibrosis: an unmet public health need. Respir Med 2014;108:955-67.
42. Cottin V. The impact of emphysema in pulmonary fibrosis. Eur Respir Rev 2013;22:153-7.
43. Lee CH, Kim HJ, Park CM, et al. The impact of combined pulmonary fibrosis and emphysema on mortality. Int J Tuberc Lung Dis 2011;15:1111-6.
44. Cottin V, Nunes H, Mouthon L, et al. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis Rheum 2011;63:295-304.
45. Kitaguchi Y, Fujimoto K, Hanaoka M, et al. Pulmonary function impairment in patients with combined pulmonary fibrosis and emphysema with and without airflow obstruction. Int J Chron Obstruct Pulmon Dis 2014;9:805-11.
46. Brillet PY, Cottin V, Letoumelin P, et al. Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests. J Radiol 2009;90:43-51.
47. Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012;141:222-31.
48. Akagi T, Matsumoto T, Harada T, et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med 2009;103:1209-15.
49. Mejía M, Carrillo G, Rojas-Serrano J, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009;136:10-5.
50. He L, Zhang W, Zhang J, et al. Diaphragmatic motion studied by M-mode ultrasonography in combined pulmonary fibrosis and emphysema. Lung 2014;192:553-61.
51. Kokuho N, Ishii T, Kamio K, et al. Diagnostic Values For Club Cell Secretory Protein (CC16) in Serum of Patients of Combined Pulmonary Fibrosis and Emphysema. COPD 2014. [Epub ahead of print].
52. Seeger W, Adir Y, Barberà JA, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 2013;62:D109-16.
53. Caminati A, Cassandro R, Harari S. Pulmonary hypertension in chronic interstitial lung diseases. Eur Respir Rev 2013;22:292-301.
54. Kwak N, Park CM, Lee J, et al. Lung cancer risk among patients with combined pulmonary fibrosis and emphysema. Respir Med 2014;108:524-30.
55. Usui K, Tanai C, Tanaka Y, et al. The prevalence of pulmonary fibrosis combined with emphysema in patients with lung cancer. Respirology 2011;16:326-31.
56. Kurashima K, Takayanagi N, Tsuchiya N, et al. The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis. Respirology 2010;15:843-8.
57. Saito H, Minamiya Y, Nanjo H, et al. Pathological finding of subclinical interstitial pneumonia as a predictor of postoperative acute respiratory distress syndrome after pulmonary resection. Eur J Cardiothorac Surg 2011;39:190-4.
58. Girard N, Marchand-Adam S, Naccache JM, et al. Lung cancer in combined pulmonary fibrosis and emphysema: a series of 47 Western patients. J Thorac Oncol 2014;9:1162-70.
59. Kishaba T, Shimaoka Y, Fukuyama H, et al. A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema. BMJ Open 2012;2.
60. Ando K, Sekiya M, Tobino K, et al. Relationship between quantitative CT metrics and pulmonary function in combined pulmonary fibrosis and emphysema. Lung 2013;191:585-91.
61. Schmidt SL, Nambiar AM, Tayob N, et al. Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema. Eur Respir J 2011;38:176-83.
62. Todd NW, Jeudy J, Lavania S, et al. Centrilobular emphysema combined with pulmonary fibrosis results in improved survival. Fibrogenesis Tissue Repair 2011;4:6.
63. Cottin V, Cordier JF, Wells AU. Centrilobular emphysema combined with pulmonary fibrosis results in improved survival: a response. Fibrogenesis Tissue Repair 2011;4:16.
64. Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005;353:2229-42.
65. Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G, Anstrom KJ, et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012;366:1968-77.
66. Kreuter M. Pirfenidone: an update on clinical trial data and insights from everyday practice. Eur Respir Rev 2014;23:111-7.
67. King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2083-92.
68. Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience from a German tertiary referral center for interstitial lung diseases. Respiration 2014;88:199-207.
69. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2071-82.