Abstract
Background and objectivesEvidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). We sought to investigate whether the involvement of mediastinal lymph nodes is associated with accelerated disease progression, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up of these patients.MethodsThis retrospective study included IPF patients referred to a single ILD centre in Italy. A consensus-based assessment of mediastinal LNE on chest CT scan was performed by two thoracic radiologists. Kaplan-Meier curves and multivariate Cox proportional hazards regression were used to assess hazard ratios for mortality and disease progression (defined as categorical FVC decline ≥10%). The annualized rates of change in functional parameters for each patient were calculated using mixed linear models.ResultsThe study population consisted of 152 IPF patients, of whom 135 (89%) received antifibrotic treatment for IPF during the study follow up. Patients having evidence of 3 or more enlarged mediastinal lymph nodes on baseline CT scan showed increased rates of mortality (HR 5.03, 95% CI 1.86–13.62, p ≤ 0.001) and significant disease progression (HR 2.99, 95% CI 1.22–7.33, p = 0.17) as compared to patients without LNE, after adjusting for GAP stage. Among 62 patients with LNE who underwent a follow up CT scan of the chest and received antifibrotic treatment, 57 (92%) maintained evidence mediastinal LNE over time.ConclusionsDiffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF.
Highlights
Background and objectivesEvidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF)
We retrospectively evaluated the impact of LNE on mortality and disease progression in a cohort of IPF patients, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up
In this study we retrospectively investigated the impact of mediastinal LNE on survival and disease behaviour in patients with IPF followed up at a tertiary interstitial lung disease (ILD) centre in Italy
Summary
Background and objectivesEvidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). The evidence of mediastinal lymphadenopathies on chest CT scans inpatients with interstitial lung disease (ILD) have drawn the attention of researchers over the years, due to the putative role played by inflammation and immunity processes in pulmonary fibrosis. The frequency of this finding has been reported in up to 70% of patients with a usual interstitial pneumonia pattern [18, 19], and in up to 58% of IPF patients [20, 21]. The study recognizes the presence of hilar/mediastinal lymphadenopathies on HRCT as one of the key inclusion criteria, based on the hypothesis that lymph node involvement may identify IPF patients with pronounced immune activation and who are more likely to respond to the investigational treatment
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