METHODS: Patients diagnosed with ILD at Singapore General Hospital were prospectively recruited and compared against 36 healthy controls. The primary outcome was transplant-free survival. Genomic DNA from peripheral blood was extracted and LTL measured using quantitative polymerase chain reaction assay (qPCR).
RESULTS: Amongst 165 patients, 37% had short LTL. There was a higher proportion of combined pulmonary fibrosis and emphysema (CPFE) patients with short LTL (n = 21, 34.4% vs n = 16, 15.4%; p < 0.001). Short LTL patients had reduced survival at 12-, 24- and 36-months and median survival of 24 months (p < 0.001) which remained significant following adjustment for smoking, GAP Stage and radiological UIP pattern (Hazard Ratio (HR), 2.74; 95%CI:1.46, 5.11; p = 0.002). They had increased respiratory-related mortality and acute exacerbation incidences. Despite similar baseline lung function, short LTL patients had a faster decline in absolute forced vital capacity (FVC) of -105.3 (95% CI: 151.4, -59.1) mL/year compared to -58.2 (95% CI: 82.9, -33.6) mL/year (p < 0.001) in normal LTL patients.
CONCLUSIONS: Short LTL correlated with increased mortality and faster lung function decline in our Singaporean ILD cohort with a magnitude similar to that in Western ILD cohorts. Further research is needed to integrate LTL assessment into clinical practice.
方法:前瞻性招募在新加坡总医院诊断为ILD的患者,并与36名健康对照进行比较。主要结果是无移植存活。提取来自外周血的基因组DNA并使用定量聚合酶链反应测定(qPCR)测量LTL。
结果:在165名患者中,37%有短LTL。短LTL合并肺纤维化和肺气肿(CPFE)患者的比例更高(n=21,34.4%vsn=16,15.4%;p<0.001)。短LTL患者在12-时生存率降低,24个月和36个月以及24个月的中位生存期(p<0.001),在调整吸烟后仍然显着,GAP阶段和放射性UIP模式(危险比(HR),2.74;95CI:1.46,5.11;p=0.002)。他们增加了与呼吸有关的死亡率和急性加重发生率。尽管基线肺功能相似,与正常LTL患者的-58.2(95%CI:82.9,-33.6)mL/年相比,短LTL患者的绝对用力肺活量(FVC)下降更快,为-105.3(95%CI:151.4,-59.1)mL/年(p<0.001).
结论:在我们的新加坡ILD队列中,短LTL与死亡率增加和肺功能下降更快相关,其幅度与西方ILD队列相似。需要进一步的研究将LTL评估整合到临床实践中。