%0 Journal Article %A Newton, Chad A. %A Oldham, Justin M. %A Ley, Brett A Anand, Vikram A Adegunsoye, Ayodeji Liu, Gabrielle A Batra, Kiran A Torrealba, Jose A Kozlitina, Julia A Glazer, Craig A Strek, Mary E. %A Wolters, Paul J. %A Noth, Imre A Garcia，白细胞端粒长度和遗传变异与间质性肺疾病进展和生存的关系MUC5B rs35705950和TOLLIP rs5743890与特发性肺纤维化(IPF)相关。在这项观察性队列研究中，我们评估了这些基因组标记物与具有自身免疫特征的间质性肺炎(IPAF, n=250)和结缔组织病相关的间质性肺疾病(CTD-ILD, n=248)患者的生存结局和疾病进展率之间的相关性。IPF (n=499)作为比较指标。IPAF和CTD-ILD患者的LTL(年龄校正后log-transform T/S均值分别为−0.05±0.29和−0.04±0.25)长于IPF患者(−0.17±0.32)。对于IPAF患者，LTL <10百分位与LTL≥10百分位相比，肺功能下降更快(−6.43% /年，−0.86% /年;p<0.0001)和更差的无移植生存(危险比2.97,95% CI 1.70-5.20;p = 0.00014)。IPAF患者的MUC5B rs35705950次要等位基因频率(MAF)更高(23.2,95% CI 18.8-28.2; p<0.0001) than controls and is associated with worse transplant-free IPAF survival (hazard ratio 1.92, 95% CI 1.18–3.13; p=0.0091). Rheumatoid arthritis (RA)-associated ILD (RA-ILD) has a shorter LTL than non-RA CTD-ILD (−0.14±0.27 versus −0.01±0.23; p=0.00055) and higher MUC5B MAF (34.6, 95% CI 24.4–46.3 versus 14.1, 95% CI 9.8–20.0; p=0.00025). Neither LTL nor MUC5B are associated with transplant-free CTD-ILD survival.LTL and MUC5B MAF have different associations with lung function progression and survival for IPAF and CTD-ILD.Leukocyte telomere length and MUC5B minor allele frequency are similar for IPAF and the combined CTD-ILD group; however, the associations between these genomic markers and clinical outcomes are different for these two types of ILD http://ow.ly/wXem30njRkg %U //www.qdcxjkg.com/content/erj/53/4/1801641.full.pdf