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. 2018 Oct 3;19(1):195.
doi: 10.1186/s12931-018-0899-4.

Characteristics of lung cancer among patients with idiopathic pulmonary fibrosis and interstitial lung disease - analysis of institutional and population data

Joo Heung Yoon  1 , Mehdi Nouraie  2 , Xiaoping Chen  2 , Richard H Zou  2 , Jacobo Sellares  2   3 , Kristen L Veraldi  2 , Jared Chiarchiaro  2 , Kathleen Lindell  2 , David O Wilson  2 , Naftali Kaminski  4 , Timothy Burns  5 , Humberto Trejo Bittar  6 , Samuel Yousem  6 , Kevin Gibson  2 , Daniel J Kass  2
Affiliations

Characteristics of lung cancer among patients with idiopathic pulmonary fibrosis and interstitial lung disease - analysis of institutional and population data

Joo Heung Yoon et al. Respir Res. .

Abstract

Background: Lung Cancer is occasionally observed in patients with Idiopathic Pulmonary Fibrosis (IPF). We sought to describe the epidemiologic and clinical characteristics of lung cancer for patients with IPF and other interstitial lung disease (ILD) using institutional and statewide data registries.

Methods: We conducted a retrospective analysis of IPF and non-IPF ILD patients from the ILD center registry, to compare with lung cancer registries at the University of Pittsburgh as well as with population data of lung cancer obtained from Pennsylvania Department of Health between 2000 and 2015.

Results: Among 1108 IPF patients, 31 patients were identified with IPF and lung cancer. The age-adjusted standard incidence ratio of lung cancer was 3.34 (with IPF) and 2.3 (with non-IPF ILD) (between-group Hazard ratio = 1.4, p = 0.3). Lung cancer worsened the mortality of IPF (p < 0.001). Lung cancer with IPF had higher mortality compared to lung cancer in non-IPF ILD (Hazard ratio = 6.2, p = 0.001). Lung cancer among IPF was characterized by a predilection for lower lobes (63% vs. 26% in non-IPF lung cancer, p < 0.001) and by squamous cell histology (41% vs. 29%, p = 0.07). Increased incidence of lung cancer was observed among single lung transplant (SLT) recipients for IPF (13 out of 97, 13.4%), with increased mortality compared to SLT for IPF without lung cancer (p = 0.028) during observational period.

Conclusions: Lung cancer is approximately 3.34 times more frequently diagnosed in IPF patients compared to general population, and associated with worse prognosis compared with IPF without lung cancer, with squamous cell carcinoma and lower lobe predilection. The causality between non-smoking IPF patients and lung cancer is to be determined.

Keywords: Idiopathic pulmonary fibrosis; Lung cancer.

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Conflict of interest statement

Ethics approval and consent to participate

The ILD registry at the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease and the Lung Cancer registry at the Hillman Cancer Center were approved by the Institutional Review Board (# 0307127, modified 0307127–12) at the University of Pittsburgh.

Consent for publication

Both registries’ data were collected with consent from individual patient at the time of enrollment throughout data collection period.

Competing interests

JHY, MN, XC, JC, KV, KL, DOW and SY have nothing to disclose.

JS reports grants from Boehringer, grants and personal fees from Roche, personal fees from Rovi, outside the submitted work. TB reports personal fees from AbbVIE and Regeneron, outside the submitted work. NK reports grant and personal fees from Biogen Idec, personal fees from MMI, non-financial support from Actelion, non-financial support from Miragen, non-financial support from Astra Zeneca, non-financial support from GSK, personal fees from Pilant, and is a consultant to Samumed, Numedii, which are outside the submitted work. NK is also a member of the Scientific Advisory Committee, the Research Advisory Forum, and the Board of the Pulmonary Fibrosis Foundation, as well as serving as Deputy Editor of Thorax and BMJ. KG reports personal fees from Bayer, outside the submitted work. DJK reports research grant funding from Regeneron, outside the submitted work.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Matching of Interstitial Lung Disease (ILD) and Cancer Center Datasets Data from the Simmons Center for ILD were cross-matched with Lung Cancer data from the Hillman Cancer Center to identify patients with and without lung cancer, Idiopathic Pulmonary Fibrosis (IPF), or non-IPF ILD. Definition of abbreviations: ILD = interstitial lung disease; IPF = idiopathic pulmonary fibrosis; LC = lung cancer
Fig. 2
Fig. 2
Cumulative incidence of lung cancer among IPF (solid, n = 31) and non-IPF ILD patients (hatched, n = 16). Definition of abbreviations: ILD = interstitial lung disease; IPF = idiopathic pulmonary fibrosis
Fig. 3
Fig. 3
Comparison of survival probabilities of IPF patients with and without co-morbid lung cancer (between the two institutional datasets). Definition of abbreviations: IPF = idiopathic pulmonary fibrosis; LC = lung cancer
See this image and copyright information in PMC

References

    1. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King TE, Jr, Kondoh Y, Myers J, Muller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schunemann HJ, Fibrosis AEJACIP. 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. doi: 10.1164/rccm.2009-040GL. - DOI - PMC - PubMed
    1. Ley B, Collard HR. Epidemiology of idiopathic pulmonary fibrosis. Clin Epidemiol. 2013;5:483–492. doi: 10.2147/CLEP.S54815. - DOI - PMC - PubMed
    1. King TE, Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW, Group AS A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083–2092. doi: 10.1056/NEJMoa1402582. - DOI - PubMed
    1. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, Cottin V, Flaherty KR, Hansell DM, Inoue Y, Kim DS, Kolb M, Nicholson AG, Noble PW, Selman M, Taniguchi H, Brun M, Le Maulf F, Girard M, Stowasser S, Schlenker-Herceg R, Disse B, Collard HR, Investigators IT. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–2082. doi: 10.1056/NEJMoa1402584. - DOI - PubMed
    1. Wuyts WA, Cavazza A, Rossi G, Bonella F, Sverzellati N, Spagnolo P. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic? Eur Respir Rev. 2014;23:308–319. doi: 10.1183/09059180.00004914. - DOI - PMC - PubMed