Article Text
Abstract
Background and Aims The interstitial lung diseases are a heterogenous group of conditions with varying degrees of inflammation and fibrosis of the lungs. Disruption of the endothelial glycocalyx (EG) and subsequent vascular remodelling has been implicated in the pathogenesis of organ fibrosis but its role in the pathogenesis of pulmonary fibrosis is unknown. We aimed to assess the acceptability of EG assessment using sublingual video-microscopy and compare EG health between patients with fibrotic-ILD and healthy controls.
Methods Patients with fibrotic-ILD and age and sex matched healthy controls were recruited to a prospective observational study (PREDICT-ILD NCT05609201). Participants with diabetes, renal disease (eGFR<60) or connective tissue disease were excluded. EG assessment was performed using sublingual side-stream darkfield video-microscopy (GlycoCheckTM). GlycoCheckTM measures perfused boundary region (PBR) in vessels 5–25μm in width, a surrogate for EG width. Acceptability was measured using a modified theoretical framework of acceptability questionnaire. Participants in the fibrotic-ILD arms underwent pulmonary function testing on the same day as EG assessment.
Results 18 patients with IPF, 18 patients with non-IPF fibrotic-ILD (11 fibrotic hypersensitivity pneumonitis, 3 unclassifiable-ILD, 3 fibrotic non-specific interstitial pneumonia and 1 asbestosis) and 16 healthy controls were recruited. There was no significant difference in age or gender between groups. There was no significant different in% predicted FVC or% predicted DLCO between IPF and non-IPF fibrotic-ILD arms (76.5% vs 68.7%, p=0.197 and 50.9% vs 47.5%, p=0.534 respectively).
EG measurement was not possible in 2 participants in the non-IPF fibrotic ILD arm due to breathing pattern and tongue movement. There was no statistically significant difference between groups in intervention comfort, burden, self-efficacy and overall acceptability (p=0.169–0.987).
Mean PBR 5–25μm was higher in the IPF group vs controls 2.12μm vs 1.99μm (p=0.019) (figure 1). There was no difference between the non-IPF fibrotic-ILD group and IPF patients (p=0.492) or controls (p=0.216). The IPF group demonstrated significant correlation between% predicted FVC and PBR (5–25) r=-0.478 p=0.045.
Conclusions Sublingual video-microscopy is an acceptable test in patients with fibrotic-ILD. Baseline results suggest there may be a decrease in sublingual endothelial glycocalyx thickness in patients with IPF compared to healthy controls.