Temporal progression of mediastinal lymphadenopathy in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease (ILD) with limited therapeutic options and poor prognosis. [1] The average life expectancy from diagnosis is 2 to 4 years [2]; however, predicting an individual patient disease trajectory is challenging and there are no established clinically available disease biomarkers [3]. Computed tomography (CT) scanning of the chest is an essential part of the diagnostic pathway in IPF with characteristic appearances of usual interstitial pneumonia (UIP) [4]. In addition, mediastinal lymphadenopathy (MLN) has been described with high prevalence (52–92%) in IPF cohorts [5–9]. Previous studies have identified that the presence of MLN is linked to disease severity and can independently predict reduced survival in IPF [10, 11] and ILD [9]. The presence of MLN has been shown to persist on longitudinal imaging in the majority of patients with IPF who have MLN at baseline [11]. However, whilst temporal progression of MLN has been shown to correlate with worsening CT fibrosis score [6], whether temporal progression of MLN in IPF confers an additional impact on mortality is unknown. In this study we investigated temporal trends in MLN and their impact on survival in patients with IPF in two independent cohorts