Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE)

Yoshiyuki Oyama, Noriyuki Enomoto*, Yuzo Suzuki, Masato Kono, Tomoyuki Fujisawa, Naoki Inui, Yutaro Nakamura, Shigeki Kuroishi, Koshi Yokomura, Mikio Toyoshima, Shiro Imokawa, Keiji Oishi, Satoshi Watanabe, Kazuo Kasahara, Tomohisa Baba, Takashi Ogura, Hiroshi Ishii, Kentaro Watanabe, Yasuhiko Nishioka, Takafumi Suda

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Background Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial pneumonia with upper lobe predominance and fibroelastosis. Although definite diagnosis requires surgical lung biopsy (SLB), SLB is often difficult because of its complications such as refractory pneumothorax. Objective To evaluate urinary desmosines (degradation product of mature elastin) as a novel biomarker in patients with PPFE. Methods Biopsy-proven patients with PPFE (n = 14) were prospectively enrolled. Levels of urinary desmosines in patients with PPFE were measured with liquid chromatography-tandem mass spectrometry (LC-MS/MS) and compared with those in patients with idiopathic pulmonary fibrosis (IPF), patients with chronic obstructive pulmonary disease (COPD), and controls. Results Levels of urinary desmosines were significantly higher in patients with PPFE than those in patients with IPF (48.4 vs. 28.6 ng/mg creatinine, p = 0.034), patients with COPD (8.0 ng/mg creatinine, p < 0.001), or controls (17.4 ng/mg creatinine, p < 0.001). Desmosines discriminated between PPFE and IPF (area under the curve [AUC] = 0.708), and between PPFE and controls (AUC = 0.956). However, levels of desmosines were not correlated with physiological parameters in patients with PPFE. Conclusions Urinary desmosines may be a useful diagnostic biomarker in patients with PPFE. Measurement of desmosines combined with specific clinical and radiological features of PPFE may lead to an accurate diagnosis without SLB in patients with PPFE.

Original languageEnglish (US)
Pages (from-to)63-70
Number of pages8
JournalRespiratory Medicine
Volume123
DOIs
StatePublished - Feb 1 2017

Keywords

  • Desmosines
  • Elastic fibre
  • Idiopathic pulmonary fibrosis
  • Pleuroparenchymal fibroelastosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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