BMC Pulmonary Medicine (Mar 2018)

Elevated α-defensin levels in plasma and bronchoalveolar lavage fluid from patients with myositis-associated interstitial lung disease

  • Noriho Sakamoto,
  • Hiroshi Ishimoto,
  • Tomoyuki Kakugawa,
  • Minoru Satoh,
  • Tomoko Hasegawa,
  • Shin Tanaka,
  • Atsuko Hara,
  • Shota Nakashima,
  • Hirokazu Yura,
  • Takuto Miyamura,
  • Hanako Koyama,
  • Towako Morita,
  • Seiko Nakamichi,
  • Yasushi Obase,
  • Yuji Ishimatsu,
  • Hiroshi Mukae

DOI
https://doi.org/10.1186/s12890-018-0609-5
Journal volume & issue
Vol. 18, no. 1
pp. 1 – 8

Abstract

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Abstract Background Interstitial lung disease (ILD) is a prognostic indicator of poor outcome in myositis. Although the pathogenesis of myositis-associated ILD is not well understood, neutrophils are thought to play a pivotal role. Neutrophils store azurophil granules that contain defensins, which are antimicrobial peptides that regulate the inflammatory response. Here, we evaluated levels of the human neutrophil peptides (HNPs) α-defensin 1 through 3 in patients with myositis-associated ILD to determine whether HNPs represent disease markers and play a role in the pathogenesis of myositis-associated ILD. Methods HNP levels were measured in the plasma and bronchoalveolar lavage fluid (BALF) of 56 patients with myositis-associated ILD and 24 healthy controls by enzyme-linked immunosorbent assay. Results Analysis revealed significantly higher HNP levels in plasma and BALF samples from patients with myositis-associated ILD as compared to those of healthy controls; however, plasma HNPs were significantly correlated with total cell counts in BALF. Additionally, BALF HNP levels were positively correlated with serum surfactant protein-A and the percentage of neutrophils in BALF, and BALF HNP levels correlated with the percentage of reticular opacities in high-resolution computed tomography results for patients with anti-aminoacyl-tRNA synthetase (ARS) antibody positive myositis-associated ILD. Survival did not differ between patients with higher and lower levels of plasma and BALF HNPs. Conclusions Plasma and BALF HNPs might reflect the disease activities of myositis-associated ILD, especially in patients with anti-ARS antibody positive myositis-associated ILD. However further studies are necessary to clarify whether HNPs represent disease markers and play roles in disease pathogenesis.

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