Risk Factors and Clinical Characteristics of Lung Cancer in Idiopathic Pulmonary Fibrosis

A Retrospective Cohort Study

Hongseok Yoo; Byeong-Ho Jeong; Myung Jin Chung; Kyung Soo Lee; O. Jung Kwon; Man Pyo Chung


BMC Pulm Med. 2019;19(149) 

In This Article


Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia, characterized by chronic progressive fibrosis of lung without etiologies.[1] Despite continuous efforts to develop therapeutic agents, no curative treatment exists for IPF. Thus, IPF is associated with poor prognosis, with median survival of 3 years following diagnosis.[2] Poor prognosis of IPF is attributable to the progressive nature of fibrosis, which is a unique characteristic of disease that leads to severe respiratory failure. However, recent studies demonstrated that complications of IPF such as acute exacerbation, coronary artery disease, pulmonary hypertension, gastroesophageal reflux disease may result in substantial mortality and morbidity.[3–7]

One of the most fatal comorbidities of IPF is lung cancer with a reported prevalence of 4.4 to 48% in patients with IPF.[8,9] Although the specific mechanism of lung cancer development in IPF is not fully understood, the increased incidence compared to patients without IPF has also been noted in large epidemiologic studies.[9–11] The significance of lung cancer in IPF lies not only in its high incidence but also in its impact on survival. Recent studies have shown that the comorbidity of lung cancer in IPF patients considerably reduces survival due to complications from treatment and from lung cancer itself.[11–13] Therefore, the need to identify predictive factors and clinical characteristics of lung cancer in IPF is essential for establishing screening protocols and diagnostic and therapeutic strategies. Despite its importance, few data on these subjects are available in the literature. Furthermore, most studies have limitations of a small number of investigated patients or analysis of a specific subset of patients; thus, they are not able to fully depict the nature of lung cancer in IPF patients.[14–16] In this study, we aimed to identify the cumulative incidence, risk factors, and clinical characteristics of lung cancer in patients during follow up of IPF.