Abstract
BackgroundLung 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.MethodsWe 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.ResultsAmong 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.ConclusionsLung 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.
Highlights
Lung Cancer is occasionally observed in patients with Idiopathic Pulmonary Fibrosis (IPF)
Patient demographics and characteristics We identified 1953 patients including 1108 IPF patients and 841 patients with non-IPF interstitial lung disease (ILD) diagnosis from the Simmons ILD data (Fig. 1)
In our study, which represents the largest review in the United States, we have found that patients with IPF exhibited a 3.34-fold higher incidence of lung cancer compared to the general population who developed lung cancer
Summary
Lung Cancer is occasionally observed in patients with Idiopathic Pulmonary Fibrosis (IPF). Yoon et al Respiratory Research (2018) 19:195 in survival rate compared to IPF without lung cancer [11,12,13,14], but a more recent study has supported a worse prognosis [15]. In this context, the purpose of our study was to compare primary lung cancers in a large cohort of patients with IPF and non-IPF ILD to lung cancers from population data in order to describe the characteristics of and to estimate the incidence and prevalence of lung cancer in patients with IPF and non-IPF ILD
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