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

- Desmosines, degradation products of mature elastin, were prospectively evaluated in patients with biopsy-proven PPFE.
- Urinary desmosines were measured with liquid chromatography-tandem mass spectrometry (LC-MS/MS).
- Levels of urinary desmosines were significantly higher in patients with PPFE than those in patients with IPF or COPD.
- 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.

BackgroundPleuroparenchymal 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.ObjectiveTo evaluate urinary desmosines (degradation product of mature elastin) as a novel biomarker in patients with PPFE.MethodsBiopsy-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.ResultsLevels 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.ConclusionsUrinary 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.