Lung cancer in patients with idiopathic pulmonary fibrosis: A retrospective multicentre study in Europe

Abstract

Background and Objective There remains a paucity of large databases for patients with idiopathic pulmonary fibrosis (IPF) and lung cancer. We aimed to create a European registry. Methods This was a multicentre, retrospective study across seven European countries between 1 January 2010 and 18 May 2021. Results We identified 324 patients with lung cancer among 3178 patients with IPF (prevalence = 10.2%). By the end of the 10 year‐period following IPF diagnosis, 26.6% of alive patients with IPF had been diagnosed with lung cancer. Patients with IPF and lung cancer experienced increased risk of all‐cause mortality than IPF patients without lung cancer (HR: 1.51, [95% CI: 1.22–1.86], p < 0.0001). All‐cause mortality was significantly lower for patients with IPF and lung cancer with a monocyte count of either <0.60 or 0.60–<0.95 K/μl than patients with monocyte count ≥0.95 K/μl (HR [<0.60 vs. ≥0.95 K/μl]: 0.35, [95% CI: 0.17–0.72], HR [0.60–<0.95 vs. ≥0.95 K/μl]: 0.42, [95% CI: 0.21–0.82], p = 0.003). Patients with IPF and lung cancer that received antifibrotics presented with decreased all cause‐mortality compared to those who did not receive antifibrotics (HR: 0.61, [95% CI: 0.42–0.87], p = 0.006). In the adjusted model, a significantly lower proportion of surgically treated patients with IPF and otherwise technically operable lung cancer experienced all‐cause mortality compared to non‐surgically treated patients (HR: 0.30 [95% CI: 0.11–0.86], p = 0.02). Conclusion Lung cancer exerts a dramatic impact on patients with IPF. A consensus statement for the management of patients with IPF and lung cancer is sorely needed.

SH2 Domain-Containing Phosphatase-SHP2 Attenuates Fibrotic Responses through Negative Regulation of Mitochondrial Metabolism in Lung Fibroblasts

“…Finally, it is also interesting that another mediator of cancer, SHP2, is a potential therapeutic target for pulmonary fibrosis [ 35 , 36 , 37 ]. Focusing on commonalities between cancer and pulmonary fibrosis might lead to novel anti-fibrotic compounds, similarly with the process of nintedanib repositioning [ 38 ].…”

B4GALT1 as a New Biomarker of Idiopathic Pulmonary Fibrosis

“…In addition, the incidence of lung cancer in IPF patients increases over time, being 3.3% 1 year after diagnosis and racing to 54.7% after 10 years. In a previous study, we demonstrated that the expression of B4GALT1 was linked to tumor development and progression [ 5 , 8 ]. Lung cancer was prevalent and exerted a dramatic survival impact on patients with IPF [ 8 ].…”

“…In a previous study, we demonstrated that the expression of B4GALT1 was linked to tumor development and progression [ 5 , 8 ]. Lung cancer was prevalent and exerted a dramatic survival impact on patients with IPF [ 8 ]. However, the expression of B4GALT1 in IPF tissue and cell lines, as well as in UIP/IPF associated with lung cancer, was never tested before.…”

“…The process leading to the transformation of fibroblasts and maintenance of their uncontrolled activity remains largely unknown [ 5 , 6 ]. The expansion of myofibroblasts within lung interstitial space may be due to exaggerated resident fibroblast proliferation, homing of circulating fibrocytes into the lung, and/or activated type II alveolar cells that migrate into the interstitial space assuming a mesenchymal phenotype, a phenomenon known as epithelial to mesenchymal transition (EMT) [ 7 , 8 ]. EMT is a dynamic, reversible phenomenon, active during embryogenesis but implicated in wound repair cancer metastasis and fibrosis [ 3 , 4 ].…”

Prognosis of IPF patients with comorbid lung cancer: Preventing immortal time bias will make the analysis more valuable

“…We have read with interest the article by Karampitsakos et al 1 The authors established a large, multinational cohort of idiopathic pulmonary fibrosis (IPF), and compared survival, the primary outcome, by specific subgroups. In this study, the authors suggested that comorbid lung cancer increased all-cause mortality in IPF patients, while the administration of antifibrotics and surgery when technically resectable decreased all-cause mortality in IPF patients with comorbid lung cancer.…”