Telomere length in patients with unclassifiable interstitial lung disease: a cohort study.

Up to 15% of patients with chronic interstitial lung disease (cILD) will remain clinically unclassifiable ( I.e. unclassifiable ILD [uILD]) despite thorough clinical evaluation and multidisciplinary team discussion (MDT) [1, 2]. This diagnostic uncertainty translates into uncertainty in expected prognosis and initial treatment approach ( e.g. immunosuppression versus anti-fibrotic medications) for patients with uILD, and it often precludes enrollment into clinical trials. Peripheral blood telomere length (TL) is a genomic biomarker that has been associated with prognosis and harm from immunosuppression in IPF [3, 4]. TL has recently been associated with idiopathic pulmonary fibrosis (IPF)-like morphologic features ( i.e. features of usual interstitial pneumonia, UIP) and reduced survival in other forms of cILD [5–7]. Whether TL demonstrates similar associations in patients with uILD is unknown, but if so, its clinical measurement could reduce diagnostic and therapeutic uncertainty by determining which patients with uILD will have an IPF-like course. The aim of this study was to determine whether TL is associated with clinical features and outcomes in a cohort of patients with uILD. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. Ley has nothing to disclose. Conflict of interest: Dr. Liu has nothing to disclose. Conflict of interest: Dr. Elicker has nothing to disclose. Conflict of interest: Dr. Henry has nothing to disclose. Conflict of interest: Dr. Vittinghoff has nothing to disclose. Conflict of interest: Dr. Golden has nothing to disclose. Conflict of interest: Dr. Jones has nothing to disclose. Conflict of interest: Dr. Wolters reports grants from Genentech, grants and personal fees from Boehringer Ingelheim, personal fees from Blade Pharmaceuticals, grants and personal fees from Pliant, outside the submitted work.