Lung cancer in patients with Idiopathic Pulmonary Fibrosis. A retrospective multicenter study

Abstract

Background: Abundant evidence supports an association between Idiopathic Pulmonary Fibrosis (IPF) and lung cancer development. Data on diagnosis and management of patients with IPF and lung cancer are still scarce.

Aim: To estimate prevalence of lung cancer in patients with IPF.

Patients and Methods: This was a retrospective multicenter study, enrolling 835 patients with IPF from eight different centers between 2009 and 2018 in Greece.

Results: We identified 88 cases of patients with IPF and lung cancer (prevalence=10.5% n=88/835, mean age±SD=71.8±7.1, 82 males, meanFVC±SD= 73.5±18.9, meanDLCO±SD= 45.3±15.6). Six patients (7%) had both lung and other type of cancer. We identified 68 cases (77.3%) of non-small cell lung cancer (32 squamous, 25 adenocarcinoma, 11 non-specified), and 13 cases (14.8%) of small cell lung cancer. An equal distribution of anatomic location of lung cancer between upper and lower lobes was observed. Lung cancer was diagnosed post IPF diagnosis (median latency time=15.9 months) in 68% of patients and synchronously in 29.3% of patients. Chemotherapy was applied in 31.8% of cases, while 33.3% of patients underwent surgery. Median survival of patients with IPF and lung cancer was 27.4 months (95% CI: 20.6 to 36.8).

Conclusions: IPF is a risk factor for lung cancer development. Squamous cell carcinoma is the most common histologic subtype in patients with IPF. Large randomized controlled studies on the management of patients with IPF and lung cancer are sorely needed.