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Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis.

Abstract：

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.

journal_name

Respir Med

journal_title

Respiratory medicine

authors

Kinoshita Y,Ikeda T,Kushima H,Fujita M,Nakamura T,Nabeshima K,Ishii H

doi

10.1016/j.rmed.2020.106077

subject

Has Abstract

pub_date

2020-09-01 00:00:00

pages

106077

eissn

0954-6111

issn

1532-3064

pii

S0954-6111(20)30217-1

journal_volume

171

pub_type

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