Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement

Abstract

Background The presence of emphysema is relatively common in patients with fibrotic interstitial lung disease. This has been designated combined pulmonary fibrosis and emphysema (CPFE). The lack of consensus over definitions and diagnostic criteria has limited CPFE research. Goals The objectives of this task force were to review the terminology, definition, characteristics, pathophysiology, and research priorities of CPFE and to explore whether CPFE is a syndrome. Methods This research statement was developed by a committee including 19 pulmonologists, 5 radiologists, 3 pathologists, 2 methodologists, and 2 patient representatives. The final document was supported by a focused systematic review that identified and summarized all recent publications related to CPFE. Results This task force identified that patients with CPFE are predominantly male, with a history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired Dl CO, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis. The committee proposes to identify CPFE as a syndrome, given the clustering of pulmonary fibrosis and emphysema, shared pathogenetic pathways, unique considerations related to disease progression, increased risk of complications (pulmonary hypertension, lung cancer, and/or mortality), and implications for clinical trial design. There are varying features of interstitial lung disease and emphysema in CPFE. The committee offers a research definition and classification criteria and proposes that studies on CPFE include a comprehensive description of radiologic and, when available, pathological patterns, including some recently described patterns such as smoking-related interstitial fibrosis. Conclusions This statement delineates the syndrome of CPFE and highlights research priorities.

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     Hashimoto N, Iwano S, Kawaguchi K, et al. Impact of Thin-Section Computed Tomography-Determined Combined Pulmonary Fibrosis and Emphysema on Outcomes Among Patients With Resected Lung Cancer. Ann Thorac Surg. 2016;102:440-7.

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201. 201.

     Maeda R, Funasaki A, Motono N, Sekimura A, Usuda K, Uramoto H. Combined pulmonary fibrosis and emphysema predicts recurrence following surgery in patients with stage I non-small cell lung cancer. Med Oncol. 2018;35:31.

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202. 202.

     Nasim F, Moua T. Lung cancer in combined pulmonary fibrosis and emphysema: a large retrospective cohort analysis. ERJ open research. 2020;6.

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203. 203.

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204. 204.

     Takenaka T, Furuya K, Yamazaki K, Miura N, Tsutsui K, Takeo S. The prognostic impact of combined pulmonary fibrosis and emphysema in patients with clinical stage IA non-small cell lung cancer. Surg Today. 2018;48:229-35.

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205. 205.

     Oh JY, Lee YS, Min KH, et al. Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema: A retrospective study. Medicine (Baltimore). 2018;97:e11683.

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206. 206.

     Moon SW, Park MS, Kim YS, et al. Combined pulmonary fibrosis and emphysema and idiopathic pulmonary fibrosis in non-small cell lung cancer: impact on survival and acute exacerbation. BMC polm. 2019;19:177.

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207. 207.

     Ikuyama Y, Ushiki A, Kosaka M, et al. Prognosis of patients with acute exacerbation of combined pulmonary fibrosis and emphysema: a retrospective single-centre study. BMC polm. 2020;20:144.

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208. 208.
     Acute exacerbations of COPD versus IPF in patients with combined pulmonary fibrosis and emphysema · 2020

     Not checked
209. 209.

     Zheng H, Yu X, Chen P, et al. Clinical features and prognosis differences of advanced pulmonary fibrosis and combined advanced pulmonary fibrosis and emphysema syndrome in the elderly. Int J Clin Exp Med. 2017;10:13808-12.

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210. 210.

     Lee SH, Park JS, Kim SY, et al. Clinical features and prognosis of patients with idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease. Int J Tuberc Lung Dis. 2019;23:678-84.

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     Zhao A, Gudmundsson E, Mogulkoc N, et al. Mortality in combined pulmonary fibrosis and emphysema patients is determined by the sum of pulmonary fibrosis and emphysema. ERJ open research. 2021;7.

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     Cottin V, Cordier JF, Wells AU. Centrilobular emphysema combined with pulmonary fibrosis results in improved survival: a response. Fibrogenesis Tissue Repair. 2011;4:16.

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     Antoniou KM, Hansell DM, Rubens MB, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med. 2008;177:190-4.

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215. 215.

     Desai SR, Wells AU, Rubens MB, du Bois RM, Hansell DM. Traction bronchiectasis in cryptogenic fibrosing alveolitis: associated computed tomographic features and physiological significance. Eur Radiol. 2003;13:1801-8.

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     Akira M, Inoue Y, Kitaichi M, Yamamoto S, Arai T, Toyokawa K. Usual interstitial pneumonia and nonspecific interstitial pneumonia with and without concurrent emphysema: thin-section CT findings. Radiology. 2009;251:271-9.

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     Walsh SL, Calandriello L, Sverzellati N, Wells AU, Hansell DM. Interobserver agreement for the ATS/ERS/JRS/ALAT criteria for a UIP pattern on CT. Thorax. 2016;71:45-51.

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     Lee G, Kim KU, Lee JW, Suh YJ, Jeong YJ. Serial changes and prognostic implications of CT findings in combined pulmonary fibrosis and emphysema: comparison with fibrotic idiopathic interstitial pneumonias alone. Acta Radiol. 2017;58:550-7.

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     Gürün Kaya A, Özyürek BA, Şahin Özdemirel T, Öz M, Erdogan Y. Prognostic Significance of Red Cell Distribution Width in Idiopathic Pulmonary Fibrosis and Combined Pulmonary Fibrosis Emphysema. Medical principles and practice: international journal of the Kuwait University, Health Science Centre. 2021;30:154-9.

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     Malli F, Papakosta D, Antoniou K, et al. Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort. ERJ open research. 2019;5.

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221. 221.

     Zeba F, Yanning W, Melek J, et al. Prognostic Significance of Pulmonary Artery to Aorta Ratio and Other CT Markers in Pulmonary Fibrosis With and Without Emphysema. Lung. 2021;199:677-80.

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222. 222.

     Marten K, Milne D, Antoniou KM, et al. Non-specific interstitial pneumonia in cigarette smokers: a CT study. Eur Radiol. 2009.

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223. 223.

     Walsh SL, Wells AU, Sverzellati N, et al. Relationship between fibroblastic foci profusion and high resolution CT morphology in fibrotic lung disease. BMC medicine. 2015;13:241.

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224. 224.

     Salvatore M, Singh A, Yip R, et al. Progression of probable UIP and UIP on HRCT. Clin Imaging. 2019;58:140-4.

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225. 225.

     Oikonomou A, Mintzopoulou P, Tzouvelekis A, et al. Pulmonary fibrosis and emphysema: Is the emphysema type associated with the pattern of fibrosis? World J Radiol. 2015;7:294-305.

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226. 226.

     Hobbs BD, de Jong K, Lamontagne M, et al. Genetic loci associated with chronic obstructive pulmonary disease overlap with loci for lung function and pulmonary fibrosis. Nat Genet. 2017;49:426-32.

     Not in our library10.1038/ng.3752

227. 227.

     Sakornsakolpat P, Prokopenko D, Lamontagne M, et al. Genetic landscape of chronic obstructive pulmonary disease identifies heterogeneous cell-type and phenotype associations. Nat Genet. 2019;51:494-505.

     Not in our library10.1038/s41588-018-0342-2

228. 228.

     Fingerlin TE, Murphy E, Zhang W, et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet. 2013;45:613-20.

     Not in our library10.1038/ng.2609

229. 229.

     Hanaoka M, Ito M, Droma Y, 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.

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230. 230.

     Armanios M. Telomerase and idiopathic pulmonary fibrosis. Mutation research. 2012;730:52-8.

     Not in our library10.1016/j.mrfmmm.2011.10.013

231. 231.

     Petrovski S, Todd JL, Durheim MT, et al. An Exome Sequencing Study to Assess the Role of Rare Genetic Variation in Pulmonary Fibrosis. Am J Respir Crit Care Med. 2017;196:82-93.

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232. 232.

     Snetselaar R, van Moorsel CHM, Kazemier KM, et al. Telomere length in interstitial lung diseases. Chest. 2015;148:1011-8.

     Not in our library10.1378/chest.14-3078

233. 233.

     Arish N, Petukhov D, Wallach-Dayan SB. The Role of Telomerase and Telomeres in Interstitial Lung Diseases: From Molecules to Clinical Implications. Int J Mol Sci. 2019;20.

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     Hoffman TW, van Moorsel CHM, Borie R, Crestani B. Pulmonary phenotypes associated with genetic variation in telomere-related genes. Curr Opin Pulm Med. 2018;24:269-80.

     Not in our library10.1097/mcp.0000000000000475

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     Ley B, Torgerson DG, Oldham JM, et al. Rare Protein-Altering Telomere-related Gene Variants in Patients with Chronic Hypersensitivity Pneumonitis. Am J Respir Crit Care Med. 2019;200:1154-63.

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236. 236.

     Juge PA, Borie R, Kannengiesser C, et al. Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis. Eur Respir J. 2017;49.

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237. 237.

     Alder JK, Chen JJL, Lancaster L, et al. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci U S A. 2008;105:13051-6.

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     Tsuji T, Aoshiba K, Nagai A. Alveolar cell senescence in patients with pulmonary emphysema. Am J Respir Crit Care Med. 2006;174:886-93.

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     Povedano JM, Martinez P, Flores JM, Mulero F, Blasco MA. Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction. Cell reports. 2015;12:286-99.

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     Kusko RL, Brothers JF, Tedrow J, et al. Integrated Genomics Reveals Convergent Transcriptomic Networks Underlying Chronic Obstructive Pulmonary Disease and Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2016;194:948-60.

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241. 241.

     Halu A, Liu S, Baek SH, et al. Exploring the cross-phenotype network region of disease modules reveals concordant and discordant pathways between chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis. Hum Mol Genet. 2019;28:2352-64.

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242. 242.

     Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155:242-8.

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     Ekstrom M, Gustafson T, Boman K, et al. Effects of smoking, gender and occupational exposure on the risk of severe pulmonary fibrosis: a population-based casecontrol study. BMJ Open. 2014;4:e004018.

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244. 244.

     Abramson MJ, Murambadoro T, Alif SM, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis in Australia: case-control study. Thorax. 2020;75:864-9.

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245. 245.

     Bellou V, Belbasis L, Evangelou E. Tobacco Smoking and Risk for Pulmonary Fibrosis: A Prospective Cohort Study From the UK Biobank. Chest. 2021;160:983-93.

     Not in our library10.1016/j.chest.2021.04.035

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     Murphy SE, Park SL, Balbo S, et al. Tobacco biomarkers and genetic/epigenetic analysis to investigate ethnic/racial differences in lung cancer risk among smokers. NPJ precision oncology. 2018;2:17.

     Not in our library10.1038/s41698-018-0057-y

247. 247.

     Sundar IK, Rahman I. Gene expression profiling of epigenetic chromatin modification enzymes and histone marks by cigarette smoke: implications for COPD and lung cancer. Am J Physiol Lung Cell Mol Physiol. 2016;311:L1245-l58.

     Not in our library10.1152/ajplung.00253.2016

248. 248.

     Ikezoe K, Hackett TL, Peterson S, et al. Small Airway Reduction and Fibrosis Is an Early Pathologic Feature of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2021;204:1048-59.

     Not in our library10.1164/rccm.202103-0585oc

249. 249.
     Role of matrix metalloproteinases in the pathogenesis of idiopathic pulmonary fibrosis · 2016

     No notice
250. 250.

     Segura-Valdez L, Pardo A, Gaxiola M, Uhal BD, Becerril C, Selman M. Upregulation of gelatinases A and B, collagenases 1 and 2, and increased parenchymal cell death in COPD. Chest. 2000;117:684-94.

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251. 251.

     Houghton AM. Matrix metalloproteinases in destructive lung disease. Matrix biology: journal of the International Society for Matrix Biology. 2015;44-46:167-74.

     Not in our library10.1016/j.matbio.2015.02.002

252. 252.

     Manicone AM, Gharib SA, Gong KQ, et al. Matrix Metalloproteinase-28 Is a Key Contributor to Emphysema Pathogenesis. Am J Pathol. 2017;187:1288-300.

     Not in our library10.1016/j.ajpath.2017.02.008

253. 253.

     Maldonado M, Salgado-Aguayo A, Herrera I, et al. Upregulation and Nuclear Location of MMP28 in Alveolar Epithelium of Idiopathic Pulmonary Fibrosis. Am J Respir Cell Mol Biol. 2018;59:77-86.

     Not in our library10.1165/rcmb.2017-0223oc

254. 254.

     Maldonado M, Buendia-Roldan I, Vicens-Zygmunt V, et al. Identification of MMP28 as a biomarker for the differential diagnosis of idiopathic pulmonary fibrosis. PLoS One. 2018;13:e0203779.

     Not in our library10.1371/journal.pone.0203779

255. 255.

     Selman M, Ruiz V, Cabrera S, et al. TIMP-1, -2, -3, and -4 in idiopathic pulmonary fibrosis. A prevailing nondegradative lung microenvironment? Am J Physiol Lung Cell Mol Physiol. 2000;279:L562-74.

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256. 256.

     Pardo A, Barrios R, Gaxiola M, et al. Increase of lung neutrophils in hypersensitivity pneumonitis is associated with lung fibrosis. Am J Respir Crit Care Med. 2000;161:1698-704.

     Not in our library10.1164/ajrccm.161.5.9907065

257. 257.

     Zuo F, Kaminski N, Eugui E, et al. Gene expression analysis reveals matrilysin as a key regulator of pulmonary fibrosis in mice and humans. Proc Natl Acad Sci U S A. 2002;99:6292-7.

     Not in our library10.1073/pnas.092134099

258. 258.

     Herrera I, Cisneros J, Maldonado M, et al. Matrix metalloproteinase (MMP)-1 induces lung alveolar epithelial cell migration and proliferation, protects from apoptosis, and represses mitochondrial oxygen consumption. J Biol Chem. 2013;288:25964-75.

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259. 259.

     Mattey DL, Nixon NB, Dawes PT. Association of circulating levels of MMP-8 with mortality from respiratory disease in patients with rheumatoid arthritis. Arthritis Res Ther. 2012;14:R204.

     Not in our library10.1186/ar4042

260. 260.

     Gilligan DM, O’Connor CM, Ward K, Moloney D, Bresnihan B, FitzGerald MX. Bronchoalveolar lavage in patients with mild and severe rheumatoid lung disease. Thorax. 1990;45:591-6.

     Not in our library10.1136/thx.45.8.591

261. 261.

     Doyle TJ, Patel AS, Hatabu H, et al. Detection of Rheumatoid Arthritis-Interstitial Lung Disease Is Enhanced by Serum Biomarkers. Am J Respir Crit Care Med. 2015;191:1403-12.

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262. 262.

     Moinzadeh P, Krieg T, Hellmich M, et al. Elevated MMP-7 levels in patients with systemic sclerosis: correlation with pulmonary involvement. Exp Dermatol. 2011;20:770-3.

     Not in our library10.1111/j.1600-0625.2011.01321.x

263. 263.

     Andersen GN, Nilsson K, Pourazar J, et al. Bronchoalveolar matrix metalloproteinase 9 relates to restrictive lung function impairment in systemic sclerosis. Respir Med. 2007;101:2199-206.

     Not in our library10.1016/j.rmed.2007.04.019

264. 264.

     Kennedy B, Branagan P, Moloney F, et al. Biomarkers to identify ILD and predict lung function decline in scleroderma lung disease or idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2015;32:228-36.

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265. 265.

     Manetti M, Guiducci S, Romano E, et al. Increased serum levels and tissue expression of matrix metalloproteinase-12 in patients with systemic sclerosis: correlation with severity of skin and pulmonary fibrosis and vascular damage. Ann Rheum Dis. 2012;71:1064-72.

     Not in our library10.1136/annrheumdis-2011-200837

266. 266.

     Vuorinen K, Myllärniemi M, Lammi L, et al. Elevated matrilysin levels in bronchoalveolar lavage fluid do not distinguish idiopathic pulmonary fibrosis from other interstitial lung diseases. Apmis. 2007;115:969-75.

     Not in our library10.1111/j.1600-0463.2007.apm_697.x

267. 267.
     MMP1 and MMP7 as Potential Peripheral Blood Biomarkers in Idiopathic Pulmonary Fibrosis · 2008

     No notice
268. 268.

     García-de-Alba C, Becerril C, Ruiz V, et al. Expression of matrix metalloproteases by fibrocytes: possible role in migration and homing. Am J Respir Crit Care Med. 2010;182:1144-52.

     Not in our library10.1164/rccm.201001-0028oc

269. 269.

     García de Alba C, Buendia-Roldân I, Salgado A, et al. Fibrocytes contribute to inflammation and fibrosis in chronic hypersensitivity pneumonitis through paracrine effects. Am J Respir Crit Care Med. 2015;191:427-36.

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270. 270.

     Andersson-Sjoland A, de Alba CG, Nihlberg K, et al. Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis. Int J Biochem Cell Biol. 2008;40:2129-40.

     Not in our library10.1016/j.biocel.2008.02.012

271. 271.

     Dupin I, Thumerel M, Maurat E, et al. Fibrocyte accumulation in the airway walls of COPD patients. Eur Respir J. 2019;54.

     Not in our library10.1183/13993003.02173-2018

272. 272.

     de Brouwer B, Drent M, van den Ouweland JMW, et al. Increased circulating desmosine and age-dependent elastinolysis in idiopathic pulmonary fibrosis. Respir Res. 2018;19:45.

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273. 273.

     Sato T, Kajikuri T, Saito Y, Chikuma M, Nagai S. Determination of desmosine in bronchoalveolar lavage fluids by time-resolved fluoroimmunoassay. Clin Chim Acta. 2008;387:113-9.

     Not in our library10.1016/j.cca.2007.09.015

274. 274.

     Kim C, Ko Y, Kim SH, et al. Urinary desmosine is associated with emphysema severity and frequent exacerbation in patients with COPD. Respirology. 2018;23:176-81.

     Not in our library10.1111/resp.13170

275. 275.

     Turino GM. Chronic Obstructive Pulmonary Disease. A Biomarker and a Potential Therapy. Ann Am Thorac Soc. 2018;15:S15-s7.

     Not in our library10.1513/annalsats.201707-568kv

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     Gregory AD, Kliment CR, Metz HE, et al. Neutrophil elastase promotes myofibroblast differentiation in lung fibrosis. J Leukoc Biol. 2015;98:143-52.

     Not in our library10.1189/jlb.3hi1014-493r

277. 277.

     Agusti A, Faner R. Lung function trajectories in health and disease. Lancet Respir Med. 2019;7:358-64.

     Not in our library10.1016/s2213-2600(18)30529-0

278. 278.

     Agusti A, Hogg JC. Update on the Pathogenesis of Chronic Obstructive Pulmonary Disease. N Engl J Med. 2019;381:1248-56.

     Not in our library10.1056/nejmra1900475

279. 279.

     Wong A, Ryerson CJ, Liang C, Cottin V. Combined pulmonary fibrosis and emphysema: a systematic review and meta-analysis. 2019.

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280. 280.

     Goddard PR, Nicholson EM, Laszlo G, Watt I. Computed tomography in pulmonary emphysema. Clin Radiol. 1982;33:379-87.

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281. 281.

     Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med. 2021;384:325-34.

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282. 282.

     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.

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283. 283.

     Agusti A, Bel E, Thomas M, et al. Treatable traits: toward precision medicine of chronic airway diseases. Eur Respir J. 2016;47:410-9.

     Not in our library10.1183/13993003.01359-2015

284. 284.

     Wongkarnjana A, Scallan C, Kolb MRJ. Progressive fibrosing interstitial lung disease: treatable traits and therapeutic strategies. Curr Opin Pulm Med. 2020;26:436-42.

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     Jacobs SS, Krishnan JA, Lederer DJ, et al. Home Oxygen Therapy for Adults with Chronic Lung Disease. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202:e121-e41.

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286. 286.

     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;16:137.

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287. 287.

     Cottin V, Crestani B, Cadranel J, et al. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2017. Version longue [French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2017 update. Full-length version]. Rev Mal Respir. 2017;34:900-68.

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288. 288.

     Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958-68.

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289. 289.

     Takahashi T, Terada Y, Pasque MK, et al. Clinical Features and Outcomes of Combined Pulmonary Fibrosis and Emphysema After Lung Transplantation. Chest. 2021.

     Not in our library10.1016/j.chest.2021.06.036

290. 290.

     Cottin V, Bonniaud P, Cadranel J, et al. [French practical guidelines for the diagnosis and management of IPF - 2021 update, short version]. Rev Mal Respir. 2022.

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291. 291.

     Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760-9.

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292. 292.

     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.

     Not in our library10.1056/nejmoa1402584

293. 293.

     King TE, 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.

     Not in our library10.1056/nejmoa1402582

294. 294.

     Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019;381:1718-27.

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295. 295.

     Behr J, Prasse A, Kreuter M, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021;9:476-86.

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296. 296.

     Maher TM, Corte TJ, Fischer A, et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med. 2020;8:147-57.

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297. 297.

     Cottin V, Azuma A, Raghu G, et al. Therapeutic effects of nintedanib are not influenced by emphysema in the INPULSIS trials. Eur Respir J. 2019;53.

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298. 298.

     Wells AU, Flaherty KR, Brown KK, et al. Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med. 2020;8:453-60.

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299. 299.

     Dong F, Zhang Y, Chi F, et al. Clinical efficacy and safety of ICS/LABA in patients with combined idiopathic pulmonary fibrosis and emphysema. Int J Clin Exp Med. 2015;8:8617-25.

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300. 300.

     Fishman A, Fessler H, Martinez F, et al. Patients at high risk of death after lungvolume-reduction surgery. N Engl J Med. 2001;345:1075-83.

     Not in our library10.1056/nejmoa11798

301. 301.

     van Geffen WH, Slebos DJ, Herth FJ, Kemp SV, Weder W, Shah PL. Surgical and endoscopic interventions that reduce lung volume for emphysema: a systemic review and meta-analysis. Lancet Respir Med. 2019;7:313-24.

     Not in our library10.1016/s2213-2600(18)30431-4

302. 302.

     Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46:903-75.

     Not in our library10.1183/13993003.01032-2015

303. 303.

     Shioleno AM, Ruopp NF. Group 3 Pulmonary Hypertension: A Review of Diagnostics and Clinical Trials. Clin Chest Med. 2021;42:59-70.

     Not in our library10.1016/j.ccm.2020.11.006

304. 304.
     Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias. · 2015

     Not checked
305. 305.

     Zimmermann GS, von Wulffen W, Huppmann P, et al. Haemodynamic changes in pulmonary hypertension in patients with interstitial lung disease treated with PDE-5 inhibitors. Respirology. 2014;19:700-6.

     Not in our library10.1111/resp.12294

306. 306.

     Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, Hunninghake GW. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010;363:620-8.

     Not in our library10.1056/nejmoa1002110

307. 307.

     Kolb M, Raghu G, Wells AU, et al. Nintedanib plus Sildenafil in Patients with Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018;379:1722-31.

     Not in our library10.1056/nejmoa1811737

308. 308.

     Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013;158:641-9.

     Not in our library10.7326/0003-4819-158-9-201305070-00003

309. 309.

     Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med. 2019;7:780-90.

     Not in our library10.1016/s2213-2600(19)30250-4

310. 310.

     Nathan SD, Cottin V, Behr J, et al. Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study. J Heart Lung Transplant. 2021;40:494-503.

     Not in our library10.1016/j.healun.2021.02.006

311. 311.

     Nathan SD, Waxman A, Rajagopal S, et al. Inhaled treprostinil and forced vital capacity in patients with interstitial lung disease and associated pulmonary hypertension: a post-hoc analysis of the INCREASE study. Lancet Respir Med. 2021;9:1266-74.

     Not in our library10.1016/s2213-2600(21)00165-x

312. 312.

     Thai AA, Solomon BJ, Sequist LV, Gainor JF, Heist RS. Lung cancer. Lancet. 2021;398:535-54.

     Not in our library10.1016/s0140-6736(21)00312-3

313. 313.

     Punjabi NM, Shade D, Patel AM, Wise RA. Measurement variability in single-breath diffusing capacity of the lung. Chest. 2003;123:1082-9.

     Not in our library10.1378/chest.123.4.1082

314. 314.

     Kaner RJ, Bajwa EK, El-Amine M, et al. Design of Idiopathic Pulmonary Fibrosis Clinical Trials in the Era of Approved Therapies. Am J Respir Crit Care Med. 2019;200:133-9.

     Not in our library10.1164/rccm.201903-0592pp

315. 315.

     Spagnolo P, Cocconcelli E, Cottin V, Meyer KC, Nathan SD. Idiopathic pulmonary fibrosis. 2019;433-53.

     Not in our library

316. 316.

     Karimi-Shah BA, Chowdhury BA. Forced vital capacity in idiopathic pulmonary fibrosis--FDA review of pirfenidone and nintedanib. N Engl J Med. 2015;372:1189-91.

     Not in our library10.1056/nejmp1500526

317. 317.

     Lowe KE, Regan EA, Anzueto A, et al. COPDGene(®) 2019: Redefining the Diagnosis of Chronic Obstructive Pulmonary Disease. Chronic obstructive pulmonary diseases (Miami, Fla). 2019;6:384-99.

     Not in our library10.15326/jcopdf.6.5.2019.0149

318. 318.

     Doyle TJ, Washko GR, Fernandez IE, et al. Interstitial lung abnormalities and reduced exercise capacity. Am J Respir Crit Care Med. 2012;185:756-62.

     Not in our library10.1164/rccm.201109-1618oc

319. 319.

     Putman RK, Hatabu H, Araki T, et al. Association Between Interstitial Lung Abnormalities and All-Cause Mortality. Jama. 2016;315:672-81.

     Not in our library10.1001/jama.2016.0518

Reads

A person counts once a day per paper. We keep no cookie and no address: a reader is a hash that changes every day. Any month under five reads shows no number.