Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 Jun 20;8(2):00115-2022.
doi: 10.1183/23120541.00115-2022. eCollection 2022 Apr.

Lung cancer in patients with fibrosing interstitial lung diseases: an overview of current knowledge and challenges

Namrata Kewalramani  1 , Carlos Machahua  1   2 , Venerino Poletti  3 , Jacques Cadranel  4 , Athol U Wells  5 , Manuela Funke-Chambour  1   2
Affiliations
Review

Lung cancer in patients with fibrosing interstitial lung diseases: an overview of current knowledge and challenges

Namrata Kewalramani et al. ERJ Open Res. .

Erratum in

Abstract

Patients with progressive fibrosing interstitial lung diseases (fILD) have increased morbidity and mortality. Lung fibrosis can be associated with lung cancer. The pathogenesis of both diseases shows similarities, although not all mechanisms are understood. The combination of the diseases is challenging, due to the amplified risk of mortality, and also because lung cancer treatment carries additional risks in patients with underlying lung fibrosis. Acute exacerbations in fILD patients are linked to increased mortality, and the risk of acute exacerbations is increased after lung cancer treatment with surgery, chemotherapy or radiotherapy. Careful selection of treatment modalities is crucial to improve survival while maintaining acceptable quality of life in patients with combined lung cancer and fILD. This overview of epidemiology, pathogenesis, treatment and a possible role for antifibrotic drugs in patients with lung cancer and fILD is the summary of a session presented during the virtual European Respiratory Society Congress in 2021. The review summarises current knowledge and identifies areas of uncertainty. Most current data relate to patients with combined idiopathic pulmonary fibrosis and lung cancer. There is a pressing need for additional prospective studies, required for the formulation of a consensus statement or guideline on the optimal care of patients with lung cancer and fILD.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest: N. Kewalramani reports grants and nonfinancial support from CSL Behring outside the submitted work. Conflict of interest: C. Machahua has nothing to disclose. Conflict of interest: V. Poletti reports personal fees from Boehringer Ingelheim, Roche, AMBU and ERBE, outside the submitted work. Conflict of interest: J. Cadranel reports fees for participation on boards of experts for the development of cancer drugs from AbbVie, AZ, BI, BMS, Jansen, MSD, Novartis, Pfizer, Roche and Takeda. Conflict of interest: A.U. Wells reports personal fees and nonfinancial support from Boehringer Ingelheim, Bayer and Roche Pharmaceuticals, and personal fees from Blade, outside the submitted work. Conflict of interest: M. Funke-Chambour reports grants from Boehringer Ingelheim and Roche, and other support from MSD, outside the submitted work.

Figures

FIGURE 1
FIGURE 1
Pathomechanisms of lung cancer and fibrosing interstitial lung disease. Dysplastic bronchial cells accumulate near fibroblastic foci. Senescence-associated secretory phenotype (SASP) and stem-cell exhaustion contribute to dysplastic bronchial cell development. Fibrotic processes including epithelial–mesenchymal transition, Wnt/β-catenin and Sonic Hedgehog pathways contribute to cancer development. Antifibrotic drugs inhibit fibrosis and might have potential effects on cancer. HSP: heat shock protein; TGF: transforming growth factor. Created with BioRender (www.biorender.com).
FIGURE 2
FIGURE 2
A proposal for adapted modern therapeutic strategies for lung cancer fibrosing interstitial lung disease (fILD) other than small cell lung carcinoma. PS: performance status; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide; NA: not available; UIP: usual interstitial pneumonia; CT: computed tomography; FVC: forced vital capacity; GAP: gender, age, physiology; VATS: video-assisted thoracoscopic surgery; SBRT: stereotactic body radiotherapy.
See this image and copyright information in PMC

References

    1. Raghu G, Remy-Jardin M, Myers JL, et al. . Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018; 198: e44–e68. doi:10.1164/rccm.201807-1255ST - DOI - PubMed
    1. Lynch JP, Saggar R, Weigt SS, et al. . Usual interstitial pneumonia. Semin Respir Crit Care Med 2006; 27: 634–651. doi:10.1055/s-2006-957335 - DOI - PubMed
    1. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med 2020; 383: 958–968. doi:10.1056/NEJMra2005230 - DOI - PubMed
    1. Collard HR, Moore BB, Flaherty KR, et al. . Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176: 636–643. doi:10.1164/rccm.200703-463PP - DOI - PMC - PubMed
    1. Miyashita K, Kono M, Saito G, et al. . Prognosis after acute exacerbation in patients with interstitial lung disease other than idiopathic pulmonary fibrosis. Clin Respir J 2021; 15: 336–344. doi:10.1111/crj.13304 - DOI - PubMed