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Background  Mediastinal lymph node enlargement (LNE) is common in idiopathic pulmonary fibrosis (IPF) and is known to be associated with the severity of lung fibrosis. However, the relationship between mediastinal LNE and the prognosis of IPF has not been determined to date.    Methods  This study included patients with IPF from the interstitial lung disease registry at Seoul National University Bundang Hospital, from January 2012 to March 2016. Two thoracic radiologists independently reviewed mediastinal LNE and lung parenchymal fibrosis and ground glass opacities in chest computed tomography scans of each patient, which were obtained upon diagnosis. Mortality and admission rates were analyzed.    Results  In total, 132 patients (104 [78.8%] male; median age, 72 years; range, 51–84 years) were enrolled and 73 (55.3%) patients had mediastinal LNE (short axis ≥ 10 mm in diameter). Mortality was significantly higher among patients with LNE than among those without LNE (hazard ratio 2.26 [95% confidence interval 1.20–4.23], p = 0.011). Of the patients with LNE, 24.7% experienced acute exacerbation and 43.8% experienced hospital admission for respiratory causes, in comparison with 16.9% and 40.0% of patients without LNE respectively. Although patients with LNE had a tendency to have increased rate of acute exacerbation, it was not statistically significant.    Conclusion  Mediastinal LNE in IPF is associated with increased mortality and its occurrence may be considered a poor prognostic factor in patients with IPF.

Impact of mediastinal lymph node enlargement on the prognosis of idiopathic pulmonary fibrosis