Mediastinal lymph node enlargement in idiopathic pulmonary fibrosis: relationships with disease progression and pulmonar

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RESEARCH ARTICLE

Open Access

Mediastinal lymph node enlargement in idiopathic pulmonary fibrosis: relationships with disease progression and pulmonary function trends Giacomo Sgalla1* , Anna Rita Larici2,3, Nicoletta Golfi1, Mariarosaria Calvello1, Alessandra Farchione2, Annemilia Del Ciello2, Francesco Varone1, Bruno Iovene1, Riccardo Manfredi2,3 and Luca Richeldi1,4

Abstract Background and objectives: Evidence 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. Methods: This 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. Results: The 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. Conclusions: Diffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF. Keywords: Idiopathic pulmonary fibrosis, Interstitial lung disease, Lymphadenopathy

* Correspondence: [email protected] 1 Dipartimento Scienze Gastroenterologiche, Endocrino-Metaboliche e Nefro-Urologiche, Unità Operativa Complessa di Pneumologia, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, Rome, Italy Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and