Background

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare idiopathic interstitial pneumonia characterized by an upper lobe-dominant interstitial increase in predominantly elastic fibers. The accumulation of cases has resulted in a refinement of the disease concept, but there are no blood biomarkers to aid in the diagnosis or prediction of a progressive phenotype among PPFE patients. Several organizers, including latent transforming growth factor-β binding protein 4 (LTBP-4), are known to be involved in elastogenesis. However, the potential of LTBP-4 as a blood biomarker for PPFE has not been investigated.

Methods

We selected cases of clinically or histologically diagnosed IPPFE (n = 20) along with idiopathic pulmonary fibrosis (IPF) patients (n = 39) and healthy controls (n = 10). We quantified the protein levels of LTBP-4 in lung tissues and serum samples.

Results

The LTBP-4 levels in lung tissue of PPFE patients were 2.16 times higher than those of IPF patients (p = 0.032). The serum concentration of LTBP-4 (pg/ml) in IPPFE was higher than that in healthy controls (1429 [154–3620] vs. 187 [56.4–490], p = 0.013). The serum concentration of LTBP-4 in IPPFE was markedly higher than that in IPF without a significant difference (1429 [154–3620] vs. 915 [491–1967], p = 0.671). In addition, a higher concentration of LTBP-4 was associated with a poor prognosis in IPPFE patients.

Conclusions

The serum concentration of LTBP-4 may aid in the diagnosis of IPPFE or the prediction of an aggressive phenotype.

中文翻译：

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血清潜在转化生长因子-β结合蛋白4作为特发性胸膜实质性纤维弹性增生的新型生物标志物。

背景

特发性胸膜实质性纤维弹性增生（IPPFE）是一种罕见的特发性间质性肺炎，其特征是主要是弹性纤维的上叶为主的间质性增加。病例的积累导致疾病概念的完善，但尚无血液生物标志物可帮助诊断或预测PPFE患者中进行性表型。已知几种组织器，包括潜在的转化生长因子-β结合蛋白4（LTBP-4），都参与了弹性发生。但是，尚未研究LTBP-4作为PPFE的血液生物标志物的潜力。

方法

我们选择了临床或组织学诊断为IPPFE（n = 20）的病例，以及特发性肺纤维化（IPF）患者（n = 39）和健康对照（n = 10）。我们量化了肺组织和血清样品中LTBP-4的蛋白质水平。

结果

PPFE患者肺组织中的LTBP-4水平是IPF患者的2.16倍（p  = 0.032）。IPPFE中的LTBP-4血清浓度（pg / ml）高于健康对照组（1429 [154–3620]与187 [56.4–490]，p  = 0.013）。IPPFE中LTBP-4的血清浓度显着高于IPF中，无显着性差异（1429 [154–3620]与915 [491–1967]，p  = 0.671）。另外，更高浓度的LTBP-4与IPPFE患者的不良预后有关。

结论

血清LTBP-4的浓度可能有助于IPPFE的诊断或侵袭性表型的预测。