Background

The course of idiopathic pulmonary fibrosis (IPF) is uncertain with variable patterns of disease progression. We sought to evaluate the prognostic utility of the WBC, a routinely performed lab test, in a well-defined cohort of outpatient IPF subjects.

Methods

We reviewed IPF patient records from two independent ILD centers (Inova Fairfax in Falls Church, VA, USA and Ege University Hospital in Izmir, Turkey) between 2007 and 2018. Demographics, CBC data, and patient outcomes were obtained. Survival differences were analyzed.

Results

There were 436 IPF outpatients in the cohort with a median WBC of 8.9 × 10⁹ cells per liter. For pragmatic purposes, patients were categorized into two groups, WBC ≥9 or WBC <9. Patients with WBC <9 had a median transplant-free survival of 50.5 months from the time of the CBC, compared to 32.4 months for those with WBC ≥9 (p < 0.0001). The association between WBC and attenuated survival remained significant after adjusting for GAP stage, steroid use, and antifibrotic use when WBC was analyzed both as a continuous (HR: 1.11; 95% CI: 1.05–1.17) and a dichotomized variable (high (WBC ≥9) vs. low (WBC <9), (HR: 1.53; 95% CI:1.09–2.15). WBC and absolute neutrophil count (ANC) were highly correlated suggesting that PMNs account for most of this association (r = 0.92).

Conclusions

Baseline WBC may impart important and readily available prognostic information in outpatients with IPF. Further studies are warranted to validate this as a potential biomarker for IPF, as well as to define the biologic basis for the association.

中文翻译：

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特发性肺纤维化患者白细胞计数与预后之间的关系。

背景

特发性肺纤维化（IPF）的病程不确定，疾病进展的方式各不相同。我们试图评估WBC（常规执行的实验室测试）在门诊IPF患者的明确定义队列中的预后效用。

方法

我们回顾了2007年至2018年之间来自两个独立的ILD中心（美国弗吉尼亚州Falls Church的Inova Fairfax和土耳其伊兹密尔的Ege大学医院）的IPF患者记录。获得了人口统计学，CBC数据和患者预后。分析了生存差异。

结果

该队列中有436名IPF门诊患者，白细胞中位数 为每升8.9×10 ^9个细胞。出于务实的目的，将患者分为两组，WBC≥9或WBC <9。从CBC开始，WBC <9的患者的中位无移植生存期为50.5个月，而WBC≥9的患者为32.4个月（p <0.0001）。在对GAP阶段，类固醇使用和抗纤维化使用进行调整后，当连续性（HR：1.11; 95％CI：1.05-1.17）和二分变量（高（WBC） ≥9）与低（WBC <9），（HR：1.53； 95％CI：1.09–2.15）。WBC和绝对中性粒细胞计数（ANC）高度相关，表明PMN占了这一关联的大部分（r = 0.92 ）。

结论

基线白细胞可以为IPF门诊患者提供重要且容易获得的预后信息。有必要进行进一步的研究，以确认它是IPF的潜在生物标志物，并确定该关联的生物学基础。