Serum Krebs von den Lungen-6: promising biomarker to differentiate CPFE from IPF Serum KL-6: Discriminate CPFE from IPF

Main Article Content

Ezgi Demirdöğen
Aslı Görek Dilektaşlı
Nilüfer Aylin Acet Öztürk
Dilek Yeşilbursa
Ferah Budak
Funda Coşkun
Alper Öztürk
Ahmet Ursavaş
Güven Özkaya
Mehmet Karadağ
Esra Uzaslan

Keywords

Combined pulmonary fibrosis emphysema, Idiopathic pulmonary fibrosis, Krebs von den Lungen-6, Surfactant protein-A, Surfactant protein-D, Pulmonary function tests, Echocardiography

Abstract

Background  


Combined pulmonary fibrosis and emphysema (CPFE) has been recognised as a phenotype of pulmonary fibrosis. We aimed to compare serum surfactant protein-A (SP-A), surfactant protein-D (SP-D) and Krebs von den Lungen-6 (KL-6) levels, functional parameters, in CPFE and  IPF (idiopathic pulmonary fibrosis) patients.


Methods


Patients diagnosed with ‘CPFE’ and ‘IPF’ were consecutively included in 6 months as two groups. The patients with connective tissue diseases are excluded.


Results          


In this study, 47 patients (41 males, 6 females) with CPFE (n = 21) and IPF (n = 26) with a mean age of 70.12 ± 8.75 were evaluated. CPFE patients were older, had more intense smoking history, had lower DLCO/VA, lower FVC, and worse six-minute walking distance than the IPF group (p=0.005, p=0.027, p=0.02, p<0.001, p=0.001, respectively). Serum KL-6 levels were higher in CPFE group compared to IPF group [264.70 U/ml (228.90-786) vs 233.60 (101.8-425.4), p<0.001]. Serum KL-6 levels of 245.4 U/ml and higher have 81% sensitivity and 73% specificity for the discrimination of CPFE from IPF.


Conclusions  


Our study has shown that serum KL-6 level is a promising biomarker to differentiate CPFE from IPF. In CPFE cases respiratory and functional parameters are worse than those of pure fibrosis cases.

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