Extract
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.
Abstract
In two separate cohorts of patients with idiopathic pulmonary fibrosis, mediastinal adenopathy was common and an increase in the size of lymphadenopathy independently predicts mortality https://bit.ly/32vEMnX
Footnotes
Author contributions: Conceptualisation of the study: M.G. Jones, K. Pontoppidan, T.J.M. Wallis and J. Jacob; data curation: K. Pontoppidan, E. Gudmundsson, C.J. Brereton, T.J.M. Wallis, F.J. Thompson, S. Battison, K. Vedwan, N. Mogulkoc, Ö.S. Unat and R. Savaş; radiological analysis: S. Battison, K. Vedwan and J. Jacob; formal analysis: T.J.M. Wallis, K. Pontoppidan, E. Gudmundsson and M.G. Jones; writing original draft: T.J.M. Wallis, K. Pontoppidan and M.G. Jones; manuscript review and editing: T.J.M. Wallis, K. Pontoppidan, M.G. Jones, F.J. Thompson, C.J. Brereton, S. Battison, K. Vedwan, B.G. Marshall, L. Richeldi, S.V. Fletcher, J. Jacob and E. Gudmundsson.
Conflict of interest: J. Jacob reports fees from Boehringer Ingelheim, Roche, NHSX and GlaxoSmithKline, unrelated to the submitted work. L. Richeldi reports fees from Biogen, Roche, ImmuneWorks, Boehringer Ingelheim, Celegene, Nitto, FibroGen, Promedior, Pliant Therapeutics, Asahi Kasei, Tora, BMS, REspiVant and CSL Behring, unrelated to the submitted work. T.J.M. Wallis, E. Gudmundsson, K. Pontoppidan, N. Mogulkoc, Ö.S. Unat, R. Savaş, K. Vedwan, S. Battison, C.J. Brereton, F.J. Thompson, B.G. Marshall, S.V. Fletcher and M.G. Jones have no competing interests to declare.
Support statement: T.J.M. Wallis receives a research fellowship from the NIHR Southampton Biomedical Research Centre. This research was funded in whole or in part by the Wellcome Trust (209553/Z/17/Z). For this project, J. Jacob and E. Gudmundsson were also supported by the NIHR UCLH Biomedical Research Centre, UK. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received September 2, 2021.
- Accepted January 16, 2022.
- Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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