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Interstitial lung disease (ILD) affects up to 40% of patients with systemic sclerosis (SSc) and is the leading cause of death.1 The risk of ILD is increased in SSc patients who are older and antitopoisomerase-1 antibody (ATA) positive.2 SSc-ILD shares features with idiopathic pulmonary fibrosis (IPF), although these two fibrosing lung diseases have different natural histories. Telomere shortening is the best-defined risk factor for IPF. In familial IPF, mutations in seven telomerase and telomere-related genes explain familial clustering in 30% of cases.3 Sporadic forms of IPF are also associated with telomere shortening in 50%–60% of cases.4 5 In IPF, short telomeres are found across cell types, which is consistent with an inherited and systemic defect in telomere maintenance.4 Previous studies of telomere length (TL) in patients with SSc have yielded conflicting results, with some studies showing longer and others shorter TL compared with age-matched controls.6 7 A major caveat in these studies is that TL was measured using techniques that are both highly variable and fail to distinguish TL among leucocyte subsets. Moreover, these studies failed to examine the impact of ILD on TL.We therefore sought to determine TL in patients with SSc and associated ILD using a clinically validated robust assay that allows accurate measurement of TL in lymphocytes and granulocytes by flow cytometry and fluorescence in situ hybridisation (flowFISH).8 9
Subjects were evaluated at the Northwestern Scleroderma Program between 2013 and 2014 and …
Handling editor Josef S Smolen
Contributors MA and JV designed the study. KL, VSH, RA and MC collected the data. RA and VSH analysed the data. MA and KL conducted statistical analyses. KL, MA and JV interpreted the data and wrote manuscript. All authors reviewed the manuscript and approved final version.
Funding This study was supported by Slovenian research agency (Z3-9261 to KL) and the Commonwealth Foundation Iniative at Johns Hopkins (to MA).
Competing interests None declared.
Patient consent for publication Obtained.
Ethics approval The study was approved by Northwestern University and Johns Hopkins Medicine Institutional Review Boards.
Provenance and peer review Not commissioned; externally peer reviewed.