ArticleSarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG2022
Serum Krebs von den Lungen-6: promising biomarker to differentiate CPFE from IPF.
Article in Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed, 10 citations in OpenAlex.
- Evaluating the diagnostic and prognostic utility of serial KL-6 measurements in connective tissue disease patients at risk for interstitial lung disease: correlations with pulmonary function tests and high-resolution computed tomography.BMC pulmonary medicine · 2024Article
- Rheumatoid arthritis presenting by combined pulmonary fibrosis and emphysema with clubbing and pulmonary hypertension: A rare case report.Clinical case reports · 2024Article
- The role of biomarkers in the diagnosis and treatment follow-up of idiopathic pulmonary fibrosis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
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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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