title: Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE).
abstract: 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.
  
  
  
  To evaluate urinary desmosines (degradation product of mature elastin) as a novel biomarker in patients with PPFE.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Oyama
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan; Health Administration Center, Hamamatsu University School of Medicine, Hamamatsu, Japan. Electronic address: norieno@hama-med.ac.jp.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Enomoto
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Yuzo
authors.initials: Y
authors.lastname: Suzuki
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Kono
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Fujisawa
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan; Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Inui
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Yutaro
authors.initials: Y
authors.lastname: Nakamura
authors.affiliation: Department of Internal Medicine, Enshu Hospital, Hamamatsu, Japan.
authors.forename: Shigeki
authors.initials: S
authors.lastname: Kuroishi
authors.affiliation: Department of Respiratory Medicine, Seirei Mikatahara General Hospital, Hamamatsu, Japan.
authors.forename: Koshi
authors.initials: K
authors.lastname: Yokomura
authors.affiliation: Department of Respiratory Medicine, Hamamatsu Rosai Hospital, Hamamatsu, Japan.
authors.forename: Mikio
authors.initials: M
authors.lastname: Toyoshima
authors.affiliation: Department of Respiratory Medicine, Iwata City Hospital, Iwata, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Imokawa
authors.affiliation: The Department of Respiratory Medicine, NHO Yamaguchi-Ube Medical Center, Ube, Japan.
authors.forename: Keiji
authors.initials: K
authors.lastname: Oishi
authors.affiliation: Department of Respiratory Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Watanabe
authors.affiliation: Department of Respiratory Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Kasahara
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Baba
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ogura
authors.affiliation: Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishii
authors.affiliation: Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Watanabe
authors.affiliation: Department of Respiratory Medicine and Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Nishioka
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Suda
mesh_terms: D000368:Aged; D015415:Biomarkers / Q000652:urine; D001706:Biopsy; D003895:Desmosine / Q000652:urine*; D003937:Diagnosis, Differential; D004547:Elastic Tissue / Q000473:pathology; D005260:Female; D005355:Fibrosis; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D029424:Pulmonary Disease, Chronic Obstructive / Q000175:diagnosis; D012372:ROC Curve; D053719:Tandem Mass Spectrometry / Q000379:methods; D014057:Tomography, X-Ray Computed
chemical_list: D015415:Biomarkers; D003895:Desmosine
keywords: Desmosines; Elastic fibre; Idiopathic pulmonary fibrosis; Pleuroparenchymal fibroelastosis