Journal Information
Vol. 56. Issue 1.
Pages 47-49 (January 2020)
Vol. 56. Issue 1.
Pages 47-49 (January 2020)
Scientific Letter
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Association between idiopathic pulmonary fibrosis and lung cancer
Asociación entre fibrosis pulmonar idiopática y neoplasia pulmonar
Blanca de Vega Sáncheza,f,
Corresponding author

Corresponding author.
, Carlos Disdier Vicentea,g, Vicente Roig Figueroaa, Rafael Lopez Castrob, Jose Maria Matillac, María Rosa Lopez Pedreirad, Maria Henar Borrego Pintadoe
a Unidad de Broncoscopias y Técnicas Pleurales, Servicio de Neumología, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
b Servicio de Oncología Médica, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
c Servicio de Cirugia Torácica, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
d Servicio de Radiología, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
e Servicio de Anatomía Patológica, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
f Grupo Emergente de Neumología Intervencionista SEPAR (GEBRYN)
g Centro de Investigación en Red Enfermedades Respiratorias(CIBERES)
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Tables (1)
Table 1. Detailed characteristics of patients with idiopathic pulmonary fibrosis and lung cancer.
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To the Editor

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive form of fibrosing interstitial pneumonia, characterized by progressive symptomatic and functional worsening, with no clearly determined etiological profile. IPF is associated with passive smoking, gastroesophageal reflux, chronic viral infections, pulmonary emphysema, and lung cancer.1,2 Lung cancer is more prevalent in these patients (as documented by clinical monitoring and post-mortem reports), with a cumulative 3-year incidence of 82%. As such, IPF is considered an independent risk factor for the development of lung cancer (LC), correlating with time accumulated since diagnosis.3–7

We are therefore looking at a progressive disease with a poor prognosis and a higher incidence of LC, in which clinical management and therapeutic decision-making are a challenge.

We report a series of 13 cases from our hospital (Table 1), included between January 2014 and December 2018. In total, 93 % of the patients were male (n=12), with a mean age of 67.9 years, and, interestingly, all participants had a history of smoking (current or former). Detailed anamnesis revealed a family history of LC in 30 % of cases, and one of the patients reported a family cluster of IPF. In total, 38 % (n=5) met radiological criteria for combined pulmonary fibrosis and emphysema (CPFE).

Table 1.

Detailed characteristics of patients with idiopathic pulmonary fibrosis and lung cancer.

  Age (years)  Date of diagnosis of interstitial pattern  Date antifibrotic treatment started  Combined pulmonary fibrosis syndrome-pulmonary emphysema  Histological strain  Clinical stage  Date of diagnosis of lung cancer  Treatment of lung cancer  Time to development of lung cancer (months)  Time from diagnosis of interstitial pattern to death (months)  Time from diagnosis of lung cancer to death (months) 
Patient 1  65  October 1995    Present  Squamous  IIIB  November 2016  Chemotherapy  253  257 
Patient 2  68  March 2007    Absent  Adenocarcinoma  —  May 2018  Symptomatic  134     
Patient 3  78  July 2011    Absent  Squamous  IA  January 2014  Left lower lobectomy  30     
Patient 4  48  April 2014  March 2015  Absent  Neuroendocrine  IIIB  June 2015  Chemotherapy  14  19 
Patient 5  63  March 2015  December 2015  Absent  Squamous  IIA  November 2016  Symptomatic  20  25 
Patient 6  85  September 2015    Present  Non-small cell  IIIA  March 2016  Symptomatic  16  10 
Patient 7  61  September 2016    Absent  Squamous  IIIB  November 2016  Chemotherapy  16  14 
Patient 8  68  October 2016    Present  Adenocarcinoma  IB  October 2016  Atypical excision     
Patient 9  63  July 2017  June 2018  Absent  Adenocarcinoma  IA  December 2017  Atypical excision     
Patient 10  67  August 2017    Absent  Squamous  IIIB  October 2017  Chemotherapy+radiation therapy 
Patient 11  64  November 2017    Present  Squamous  IV  December 2017  Symptomatic 
Patient 12  80  July 2018    Present  Not known  IIIB  July 2018  Symptomatic     
Patient 13  73  September 2018      Squamous  IIIA  September 2018  Chemotherapy     

In our series, the most common tumor lineage was squamous (n=9), followed by adenocarcinoma (n=3) at rates similar to those reported in the literature.4,5,8 However, no statistical association was found between the higher incidence of peripheral lesions and their location in lower lobes, as previously described by Kwak et al.9 (p>0.05). IPF was diagnosed using clinical/radiological criteria10 (n=8) or lung histology showing a pattern of usual interstitial pneumonia (n=7).

The diagnosis of LC was synchronous with IPF and incidental in 3 cases; this figure was much lower than that reported by Huddad and Massaro.5 In the remaining patients (n=10), the presence of lung cancer was detected after a mean follow-up of 18.2 months (SD: 78.56). Clinical disease staging in our series was as follows: IA (n=2), IB (n=1), IIA (n=1), IIIA (n=2), IIIB (n=5), stage IV (n=1), and 1 patient refused complete tumor staging. These findings diverge from the high incidence of early stage lung cancer described in other series.4,8

All patients were evaluated by the thoracic tumors committee of our hospital. Their final decision was surgical treatment in 3 cases, oncological treatment in 5 (4 chemotherapy and 1 chemotherapy and radiation therapy), and symptomatic treatment in 5 patients, in view of their functional status. At a second evaluation, patients undergoing surgical treatment (after a minimum disease-free period of 6 months) were assessed by the interstitial diseases committee, and treatment with antifibrotic drugs started in 66 % (pirfenidone in all cases). The other patients continued close clinical and functional monitoring, given their absence of symptoms. None of the patients who underwent surgical treatment presented IPF exacerbations and/or postoperative complications, despite thoracic surgery being one of the possible etiologies of acute exacerbation.11 No statistically significant differences were found in the histological LC type or in the stage at diagnosis in the subgroup of CPFE patients.

Taking into account the limitations of the study, such as the differing follow-up times due to the low incidence of cases, 54 % of patients in the series have now died (n=7). In the group of patients who died, the average survival from diagnosis of IPF was 9 months (SD: 80.54). This figure was higher in patients who received antifibrotics (mean survival 22 months). Mean survival from diagnosis of LC was 10.5 months (SD: 11.39), but 66 % (n=2) of the subgroup of patients who underwent surgical treatment are still alive; the only patient from this group who died had a survival of 39 months. The subanalysis of the CPFE group revealed a similar overall mortality (60 %) with an average survival after LC diagnosis of 5.3 months (SD: 3.68).

In our series, as in other previously described cohorts, IPF appears to be the determining factor in life expectancy,8,12 and the option of surgery must be considered in selected cases. An appropriate, detailed functional assessment of the patient is essential to optimize treatment on an individual basis. We firmly believe in the need for thoracic tumor and interstitial pathology committees to work together to design a joint approach.

Although these results must be interpreted with caution, they suggest that radiological monitoring required for diagnosing IPF could include the early detection of LC, and perhaps this previously anecdotal association will become an increasingly common finding in clinical practice. The need for specific personalized radiological monitoring by tomography aimed at detecting early stage LC in this selected population must be evaluated.


As it is impossible to grant authorship to everyone, we would like to express in writing in this publication our appreciation of all the members of the Thoracic Oncology Committee of the Hospital Clínico de Valladolid and the Interstitial Diseases Committee of the Hospital Clínico de Valladolid.

American Thoracic Society, European Respiratory Society.
American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS).
Am J Respir Crit Care Med, 161 (2000), pp. 646-664
Diagnosis of Idiopathic Pulmonary Fibrosis An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
Am J Respir Crit Care Med., 198 (2018), pp. e44-e68
Y. Ozawa, T. Suda, T. Naito, N. Enomoto, D. Hashimoto, T. Fujisawa, et al.
Cumulative incidence of and predictivefactors for lung cancer in IPF.
Respirology, 14 (2009), pp. 723-728
S. Tomassetti, C. Gurioli, J.H. Ryu, P.A. Decker, C. Ravaglia, P. Tantalocco, et al.
(2015), pp. 157-164
R. Huddad, D. Massaro.
Idiopathic diffuse interstitial pulmonary fibrosis (fibrosing alveolitis), atypical epithelial proliferation and lung cancer.
Am J Med, 45 (1968), pp. 211-219
I. Le Jeune, J. Gribbin, J. West, C. Smith, P. Cullinan, R. Hubbard.
The incidence of cancer in patients with idiopathic pulmonary fibrosis and sarcoidoss in the UK.
Respir Med, 101 (2007), pp. 2534-2540
R. Hubbard, A. Venn, S. Lewis, J. Britton.
Lung cancer and cryptogenic fibrosing alveolitis a population-based cohort study.
Am J Respir Crit Care Med., 161 (2000), pp. 5-8
T. Lee, J.Y. Park, H.Y. Lee, Y.J. Cho, H.I. Yoon, J.H. Lee, et al.
Lung cancer in patients with idiopathicpulmonary fibrosis: Clinical characteristics and impact on survival.
Respiratory Medicine, 108 (2014), pp. 1549-1555
N. Kwak, C.M. Park, J. Lee, Y.S. Park, S.M. Lee, J.J. Yim, et al.
Lung cancer risk among patients with combined pulmonary fibrosis and emphysema.
Respiratory Medicine, 108 (2014), pp. 524-530
G. Raghu, H.R. Collard, J.J. Egan, F.J. Martinez, J. Behr, K.K. Brown, 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., 183 (2011), pp. 788-824
D.S. Kim, J.H. Park, B.K. Park, J.S. Lee, A.G. Hicholson, T. Colby.
Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features.
Eur Respir J., 27 (2006), pp. 143-150
T. Fujimoto, T. Okazaki, T. Matsukura, T. Hanawa, N. Yamashita, K. Nishimura, et al.
Operation for lung cancer in patients with idiopathic pulmonary fibrosis: surgical contraindication?.
Ann Thorac Surg, 76 (2003), pp. 1674-1678

Please cite this article as: Sánchez BdV, Vicente CD, Figueroa VR, Castro RL, Matilla JM, Pedreira MRL, et al. Asociación entre fibrosis pulmonar idiopática y neoplasia pulmonar. Arch Bronconeumol. 2020;56:47–49.

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