背景:特发性胸膜实质纤维弹性增生症(iPPFE),进行性纤维化疾病,特征是上叶占优势的肺纤维化,涉及胸膜和胸膜下肺实质。然而,目前尚无针对这种情况的预后标志物。已经报道了特发性肺纤维化患者的血白细胞水平与死亡率之间的关联;因此,我们假设iPPFE患者外周血白细胞水平与死亡风险相关.
方法:这项回顾性研究纵向评估了两组127例iPPFE患者在诊断时和诊断后1年的外周血白细胞计数(Seirei和Hamamatsu队列中的69例和58例患者,分别)。
结果:对外周血白细胞的综合评估显示,校正年龄后,中性粒细胞-淋巴细胞比率(NLR)与iPPFE患者的死亡率相关,多变量分析中的性别和强迫肺活量(调整后的HR,1.131;95%CI,1.032至1.227)。当根据NLR中位数对患者进行分类时,NLR高的人比NLR低的人生存期短(中位数,32.2vs79.8个月;HR,2.270;95%CI,1.416至3.696)。有趣的是,NLR中位数分类的结果在>70%的患者中纵向保留,NLR持续高的患者比其他患者死亡风险更高(中位数,24.8vs79.6个月;HR,3.079;95%CI,1.878至5.031)。与性别-年龄-生理模型相比,包含年龄的复合模型,性别和NLR可以成功地将iPPFE患者根据死亡风险分为三组.
结论:外周白细胞计数的评估很容易,可能有助于评估iPPFE患者的疾病严重程度和死亡风险。我们的研究表明,在日常实践中关注周围白细胞水平的重要性。
Idiopathic pleuroparenchymal fibroelastosis (iPPFE), a progressive fibrotic disease, is characterised by upper lobe-dominant lung fibrosis involving the pleura and subpleural lung parenchyma. However, no prognostic markers have been established for this condition. Associations between blood leucocyte levels and mortality have been reported in patients with idiopathic pulmonary fibrosis; therefore, we hypothesised that peripheral leucocyte levels are associated with mortality risk in patients with iPPFE.
This retrospective study longitudinally assessed peripheral leucocyte counts at the time of diagnosis and 1 year after diagnosis in two cohorts of 127 patients with iPPFE (69 and 58 patients in Seirei and Hamamatsu cohorts, respectively).
A comprehensive assessment of peripheral leucocytes revealed that the neutrophil-lymphocyte ratio (NLR) was associated with mortality in patients with iPPFE after adjusting for age, sex and forced vital capacity in multivariate analyses (adjusted HR, 1.131; 95% CI, 1.032 to 1.227). When the patients were classified based on the median NLR, those with a high NLR had shorter survival than those with a low NLR (median, 32.2 vs 79.8 months; HR, 2.270; 95% CI, 1.416 to 3.696). Interestingly, the results of the NLR classification by median were longitudinally preserved in >70% of patients, and patients with consistently high NLR were at a higher risk of mortality than others (median, 24.8 vs 79.6 months; HR, 3.079; 95% CI, 1.878 to 5.031). Compared with the gender-age-physiology model, a composite model comprising age, sex and NLR could successfully stratify patients with iPPFE into three groups according to mortality risk.
The assessment of peripheral leucocyte counts is easy and might be useful in evaluating disease severity and mortality risk in patients with iPPFE. Our study suggests the importance of focusing on peripheral leucocyte levels in daily practice.