Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor
DOI:
https://doi.org/10.17140/PRRMOJ-4-135Keywords:
Emphysema, Idiopathic pulmonary fibrosis, Prognosis, Pulmonary function test, Surfactant protein-DAbstract
Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is a chronic disease of unknown aetiology and is often associated with a syndrome called combined pulmonary fibrosis and emphysema (CPFE). This study aimed to identify practical predictors of prognosis in IPF patients associated with CPFE.
Subjects and Methods: We retrospectively studied 72 patients with IPF and evaluated the threshold of emphysematous area affecting prognosis on high-resolution computed tomography (HRCT) scans. As predictor candidates, various pulmonary function tests (PFTs) and biomarkers, e.g. surfactant protein (SP)-A and SP-D, were assessed.
Results: The survival rate of the CPFE group, defined as having an emphysematous area greater than 25% on HRCT, was significantly worse than that of the non-CPFE group, despite no significant difference in fibrosis scores. An annual percent decline of diffusing capacity of the lung for carbon monoxide (% DLCO) of more than 5% was a significant prognostic factor in the CPFE group. High concentration of serum SP-D was a significant prognostic factor in both the CPFE and non-CPFE groups. However, cut-off levels in the CPFE group were lower than those in the non-CPFE group.
Conclusions: We demonstrated worse prognosis in IPF associated with CPFE syndrome compared to the other subset of IPF, and showed that % DLCO and SP-D are useful predictors of poor prognosis.
References
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. doi: 10.1183/09031936.05.
Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009; 136(1): 1-2. doi: 10.13 78/chest.09-0538
Cottin V. The impact of emphysema in pulmonary fibrosis. Eur Respir Rev. 2013; 22: 153-157. doi: 10.1183/09059180.00000813
King TE Jr, Tooze JA, Schwarz MI, Brown KR, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis: Scoring system and survival model. Am J Respir Crit Care Med. 2001; 164(7): 1171-1181. doi: 10.1164/ajrccm.164.7.2003140
Jeon K, Chung MP, Lee KS, et al. Prognostic factors and causes of death in Korean patients with idiopathic pulmonary fibrosis. Respir Med. 2006; 100(3): 451-457. doi: 10.1016/j. rmed.2005.06.013
Fernandez Perez ER, Daniels CE, et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: A population-based study. Chest. 2010; 137(1): 129-137. doi: 10.1378/chest.09-1002
Natsuizaka N, Chiba H, Kuronuma K, et al. Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences. Am J Respir Crit Care Med. 2014; 190(7): 773-779. doi: 10.1164/rccm.201403- 0566OC
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(1): 10-15. doi: 10.1378/chest.08-2306
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. doi: 10.1378/chest.12-2403
Sugino K, Nakamura Y, Ito T, Isshiki T, Sakamoto S, Homma S. Comparison of clinical characteristics and outcomes between combined pulmonary fibrosis and emphysema associated with usual interstitial pneumonia pattern and non-usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2015; 32(2): 129-137. Web site. http://mattioli1885journals.com/index.php/ sarcoidosis/article/view/3470. Accessed
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. doi: 10.1111/ resp.12207
Collard HR, King TE Jr, Bartelson BB, Vourlekis JS, Schwarz MI, Brown KK. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003; 168(5): 538-542. doi: 10.1164/ rccm.200211-1311OC
Flaherty KR, Mumford JA, Murray S, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003; 168(5): 543-548. doi: 10.1164/rccm.200209-1112OC
Latsi PI, du Bois RM, Nicholson AG, et al. Fibrotic idiopathic interstitial pneumonia: The prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003; 168(5): 531-537. doi: 10.1164/rccm.200210-1245OC
Travis WD, Costabel U, Hansell DM, et al. ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013; 188(6): 733-748. doi: 10.1164/ rccm.201308-1483ST
Takahashi H, Fujishima T, Koba H, et al. Serum surfactant proteins A and D as prognostic factors in idiopathic pulmonary fibrosis and their relationship to disease extent. Am J Respir Crit Care Med. 2000; 162(3 pt 1): 1109-1114. doi: 10.1164/ ajrccm.162.3.9910080
Greene KE, King TE Jr, Kuroki Y, et al. Serum surfactant proteins-A and -D as biomarkers in idiopathic pulmonary fibrosis. Eur Respir J. 2002; 19(3): 439-446. doi: 10.1183/09031936.02.00081102
Takahashi H, Shiratori M, Kanai A, Chiba H, Kuroki Y, Abe S. Monitoring markers of disease activity for interstitial lung diseases with serum surfactant proteins A and D. Respirology. 2006; 11: S51-S54. doi: 10.1111/j.1440-1843.2006.00809.x
Yokoyama A, Kondo K, Nakajima M, et al. Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis. Respirology. 2006; 11(2): 164-168. doi: 10.1111/j.1440-1843.2006.00834.x
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. doi: 10.1164/rccm.2009- 040GL
Hanak V, Golbin JM, Hartman TE, Ryu JH. High-resolution CT findings of parenchymal fibrosis correlate with prognosis in hypersensitivity pneumonitis. Chest. 2008; 134(1): 133-138. doi: 10.1378/chest.07-3005
du Bois RM, Weycker D, Albera C, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: Test properties and minimal clinically important difference. Am J Respir Crit Care Med. 2011; 184(12): 1382-1389. doi: 10.1164/ rccm.201105-0840OC
Papiris SA, Triantafillidou C, Manali ED, et al. Combined pulmonary fibrosis and emphysema. Expert Rev Respir Med. 2013; 7(1): 19-31. doi: 10.1586/ers.12.80
Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: A review. Chest. 2012; 141(1): 222- 231. doi: 10.1378/chest.11-1062
Portillo K, Morera J. Combined pulmonary fibrosis and emphysema syndrome: A new phenotype within the spectrum of smoking-related interstitial lung disease. Pulm Med. 2012: 2012; 867870. doi: 10.1155/2012/867870
Farkas L, Gauldie J, Voelkel NF, Kolb M. Pulmonary hypertension and idiopathic pulmonary fibrosis: A tale of angiogenesis, apoptosis, and growth factors. Am J Respir Cell Mol Biol. 2011; 45(1): 1-15. doi: 10.1165/rcmb.2010-0365TR
Andersen KH, Iversen M, Kjaergaard J, et al. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease. J Heart Lung Transplant. 2012; 31(4): 373-380. doi: 10.1016/j. healun.2011.11.020
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(1): 176-183. doi: 10.1183/09031936.00114010
Akagi T, Matsumoto T, Harada T, et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med. 2009; 103(8): 1209-1215. doi: 10.1016/j.rmed.2009.02.001
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(5): 814-820. doi: 10.1111/j.1440-1843.2012.02182.x
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(5): 843- 848. doi: 10.1111/j.1440-1843.2010.01778.x
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