Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis - 09/09/20
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.
Le texte complet de cet article est disponible en PDF.Highlights |
• | LTBP-4 levels in lung tissue of IPPFE patients were higher than in IPF patients. |
• | Serum LTBP-4 levels in IPPFE patients were higher than in healthy controls. |
• | A higher serum LTBP-4 level was associated with a poor prognosis in IPPFE patients. |
Keywords : Idiopathic pulmonary fibrosis, Elastic fibers, Elastogenesis, Krebs von den Lungen-6, Elastosis
Abbreviations : BMI, FVC, HRCT, IPF, IPPFE, KL-6, LDH, LTBP-4, MCP-1, RV, SDS-PAGE, SP-A, SP-D, TBS, TBS-T, TLC, UIP
Plan
Vol 171
Article 106077- septembre 2020 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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