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Idiopathic pulmonary fibrosis and lung cancer: future directions and challenges.

ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a progressive disease of pulmonary scarring. New treatments slow disease progression and allow pulmonary fibrosis patients to live longer. Persistent pulmonary fibrosis increases a patient's risk of developing lung cancer. Lung cancer in patients with IPF differs from cancers that develop in the non-fibrotic lung. Peripherally located adenocarcinoma is the most frequent cell type in smokers who develop lung cancer, while squamous cell carcinoma is the most frequent in pulmonary fibrosis. Increased fibroblast foci in IPF are associated with more aggressive cancer behaviour and shorter doubling times. Treatment of lung cancer in fibrosis is challenging because of the risk of inducing an exacerbation of fibrosis. In order to improve patient outcomes, modifications of current lung cancer screening guidelines in patients with pulmonary fibrosis will be necessary to avoid delays in treatment. 2-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) computed tomography (CT) imaging can help identify cancer earlier and more reliably than CT alone. Increased use of wedge resections, proton therapy and immunotherapy may increase survival by decreasing the risk of exacerbation, but further research will be necessary.

SUBMITTER: Abu Qubo A

PROVIDER: S-EPMC9973524 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature

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Idiopathic pulmonary fibrosis and lung cancer: future directions and challenges.

Abu Qubo Ahmad A Numan Jamil J Snijder Juan J Padilla Maria M Austin John H M JHM Capaccione Kathleen M KM Pernia Monica M Bustamante Jean J O'Connor Timothy T Salvatore Mary M MM

Breathe (Sheffield, England) 20221201 4

Idiopathic pulmonary fibrosis (IPF) is a progressive disease of pulmonary scarring. New treatments slow disease progression and allow pulmonary fibrosis patients to live longer. Persistent pulmonary fibrosis increases a patient's risk of developing lung cancer. Lung cancer in patients with IPF differs from cancers that develop in the non-fibrotic lung. Peripherally located adenocarcinoma is the most frequent cell type in smokers who develop lung cancer, while squamous cell carcinoma is the most ...[more]

PMID: 36865932

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Project description:BackgroundCombined pulmonary fibrosis and emphysema (CPFE) patients generally have a poorer prognosis compared to those with either pulmonary fibrosis or chronic obstructive pulmonary disease (COPD)/emphysema alone, as demonstrated by several studies. Our study aimed to identify undiagnosed CPFE cases within a lung transplantation cohort initially diagnosed with interstitial lung fibrosis or COPD/emphysema. It was hypothesized that the patient cohort might contain overlooked CPFE cases, and CPFE patients would exhibit a higher incidence of pre-transplant pulmonary hypertension and post-transplant chronic lung allograft dysfunction (CLAD).MethodsThis is a single-center, retrospective cohort study conducted at the University Hospital of Zurich, Switzerland. Expert review of pre-transplant computed tomography (CT) scans by a thoracic radiologist aimed to identify undiagnosed CPFE cases. Diagnostic criteria followed established guidelines, requiring both emphysema and pulmonary fibrosis on CT imaging. A total of 133 consecutive adult bilateral lung transplantations were performed for patients with interstitial lung disease, emphysema, or both (January 1, 2013, to December 31, 2021). A total of 113 patients could be analyzed, 20 patients were excluded because of missing data. The study analyzed pulmonary function tests, pre-transplant pulmonary hypertension screening, and CLAD staging in CPFE and non-CPFE patients. Primary outcome was the incidence of CPFE among lung transplantation candidates initially diagnosed with interstitial lung fibrosis or COPD/emphysema. Secondary outcomes were comparison of pulmonary function, diffusing capacity, invasively measured pulmonary hypertension, and CLAD stages between CPFE and non-CPFE patients.ResultsBased on pre-transplant CT scans, out of 113 patients, 12 (10.6%) were reclassified as previously undiagnosed CPFE, 49 (43.4%) as non-CPFE fibrosis, and 50 (44.2%) as non-CPFE emphysema. A solitary patient (0.9%) was classified as having both fibrosis and emphysema but did not meet the criteria of CPFE. One additional patient (n=1, 0.9%) exhibited a tree-in-bud pattern. Prior to the radiological review, none of the patients had been classified as CPFE and had instead received a radiological diagnosis of COPD, emphysema, sarcoidosis, interstitial pulmonary fibrosis (IPF) or hypersensitivity pneumonitis (HP).ConclusionsThe study confirmed undiagnosed CPFE cases in 12 (10.6%) in the lung transplantation cohort, suggesting potential underdiagnosis or misclassification. Objective analysis revealed similarities in lung function, diffusing capacity, pulmonary hypertension and CLAD between CPFE and non-CPFE groups. Further exploration is warranted to understand CPFE's diagnostic nuances and clinical implications in lung transplantation.

Dataset Information

Idiopathic pulmonary fibrosis and lung cancer: future directions and challenges.

Publications

Idiopathic pulmonary fibrosis and lung cancer: future directions and challenges.

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