Characteristics of lung cancer among patients with idiopathic pulmonary fibrosis and interstitial lung disease – analysis of institutional and population data

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.Electronic supplementary materialThe online version of this article (10.1186/s12931-018-0899-4) contains supplementary material, which is available to authorized users.

Impact of idiopathic pulmonary fibrosis on recurrence after surgical treatment for stage I–III non-small cell lung cancer

“…Surgery-related AE-IPF or ARDS was also identified as an independent risk factor for mortality. Epidemiological studies have revealed that IPF is an independent risk factor for LC development [4,6,11,23], although the pathogenesis connecting the two diseases remains poorly understood. Common pathways for the development of LC in IPF, yet with distinct roles in the pathogenesis of both conditions, include genetic and epigenetic alterations; abnormal…”

“…Moreover, the relative risk of LC development is approximately eight times higher in patients with IPF than in the general population [9]. Although adenocarcinoma is the most common histopathological type of LC in the general population, patients with IPF are most commonly affected by squamous cell carcinoma, followed by adenocarcinoma [8,10,11].…”

Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme

“…IPF is a risk factor for lung cancer [92][93][94]. The reported prevalence of lung cancer in patients with IPF varies from 3-48%, and it is associated with shorter survival times than IPF alone [94][95][96].…”

“…Nintedanib is known to hinder angiogenesis, which is essential for tumour growth, metastasis and progression [77,98]. Nintedanib, therefore, has theoretical potential in the treatment of lung cancer associated with IPF, although the most common histotype of lung cancer in IPF is squamous cell carcinoma, and nintedanib is indicated for adenocarcinoma [93,94,97,98].…”

Identification of common signatures in idiopathic pulmonary fibrosis and lung cancer using gene expression modeling

“…Although the exact mechanisms remain largely unknown, it is widely accepted that genetic and environmental factors leading to alveolar epithelial cell injury trigger the repair process and induce the formation of fibroblast foci, ultimately causing pulmonary fibrosis [3]. IPF is considered as a precancerous lung disorder because occasionally patients with IPF have concomitant lung cancer, and patients with IPF have a 3.34-fold greater risk of developing primary lung cancer than the general population [4,5].…”