Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Apr 20:e13242.
doi: 10.1111/eci.13242. Online ahead of print.

Serum KL-6 levels in pulmonary Langerhans' cell histiocytosis

Miriana d'Alessandro  1 , Laura Bergantini  1 , Paolo Cameli  1 , Nicola Lanzarone  1 , Maria Antonietta Mazzei  2 , Valerio Alonzi  1 , Piersante Sestini  1 , Elena Bargagli  1
Affiliations

Serum KL-6 levels in pulmonary Langerhans' cell histiocytosis

Miriana d'Alessandro et al. Eur J Clin Invest. .

Abstract

Background: Serum Krebs von den Lungen-6 (sKL-6) is an high-molecular-weight (200 kDa) glycoprotein predominantly expressed by damaged alveolar type II cells, and it has been proposed as a potential biomarker of different ILD. This is a prognostic biomarker for chronic hypersensitivity pneumonitis (cHP) and idiopathic pulmonary fibrosis (IPF), two diseases that share several clinical and radiological features. Little data are available on the potential role of KL-6 in granulomatous and cystic interstitial lung diseases, including the orphan disease known as pulmonary Langerhans cell histiocytosis (PLCH).

Methods: For the first time, sKL-6 concentrations were assayed and compared in 96 patients (17 PLCH, 22 IPF, 34 cHP) and 22 healthy controls.

Results: Serum KL-6 concentrations were significantly higher in PLCH (599 ± 594 U/mL), IPF (1645 ± 846 U/mL) and cHP patients (1691 ± 1643 U/mL) than in healthy controls (268 U/mL) (P = .037). Area-under-the-curve values of sKL-6 were 73.4% between PLCH and healthy controls, 84.5% between IPF and PLCH and 78% between cHP and PLCH. An indirect correlation between sKL-6 concentrations and peripheral CD1a-positive cells was demonstrated (r = -0.82; P = .034).

Conclusion: Serum KL-6 concentrations were higher in PLCH patients than in controls, reflecting the alveolar damage typical of this rare interstitial lung disease.

Keywords: KL-6; chronic hypersensitivity pneumonitis; idiopathic pulmonary fibrosis; pulmonary langerhans cell histiocytosis.

PubMed Disclaimer

References

REFERENCES

    1. Inoue Y, Barker E, Daniloff E et al Pulmonary epithelial cell injury and alveolar-capillary permeability in berylliosis. Am J Respir Crit Care Med. 1997;156:109-115.
    1. Ishii H, Mukae H, Kadota J et al High serum concentrations of surfactant protein A in usual interstitial pneumonia compared with non-specific interstitial pneumonia. Thorax. 2003;58:52-57.
    1. Wakamatsu K, Nagata N, Kumazoe H et al Prognostic value of serial serum KL-6 measurements in patients with idiopathic pulmonary fibrosis. Respir Investig. 2017;55:16-23.
    1. Ohshimo S, Ishikawa N, Horimasu Y et al Baseline KL-6 predicts increased risk for acute exacerbation of idiopathic pulmonary fibrosis. Respir Med. 2014;108:1031-1039.
    1. Yokoyama A, Kondo K, Nakajima M et al Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis. Respirology. 2006;11:164-168.

LinkOut - more resources