Abstract

Background: Declining lung function signifies disease progression in idiopathic pulmonary fibrosis (IPF). Vascular endothelial growth factor (VEGF) concentration is associated with declining lung function in 6 and 12-month studies. Alveolar nitric oxide concentration (CANO) is increased in patients with IPF, however its significance is unclear. This study investigated whether baseline plasma VEGF concentration and CANO are associated with disease progression or mortality in IPF. Methods: 27 IPF patients were studied (maximum follow-up 65 months). Baseline plasma VEGF concentration, CANO and pulmonary function tests (PFTs) were measured. PFTs were performed the preceding year and subsequent PFTs and data regarding mortality were collected. Disease progression was defined as one of: death, relative decrease of ≥10% in baseline forced vital capacity (FVC) % predicted, or relative decrease of ≥15% in baseline single breath diffusion capacity of carbon monoxide (TLCO-SB) % predicted. Results: Plasma VEGF concentration was not associated with progression-free survival or mortality. There was a trend towards shorter time to disease progression and death with higher CANO. CANO was significantly higher in patients with previous declining versus stable lung function. Conclusion: The role of VEGF in IPF remains uncertain. It may be of value to further investigate CANO in IPF.

Highlights

  • Idiopathic pulmonary fibrosis (IPF) is a progressive disease characterised by irreversible distortion of the lung architecture [1] with histological changes including honeycombing, sparse cellular inflammation and fibroblastic foci [2]

  • Given the desire to determine better methods of predicting prognosis, our study aimed to explore whether plasma Vascular endothelial growth factor (VEGF) concentration or concentration of NO (CANO) are associated with progression-free survival or mortality in idiopathic pulmonary fibrosis (IPF) over a 5 year period

  • We did find that mean CANO was significantly higher in patients with previous declining versus stable lung function

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Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a progressive disease characterised by irreversible distortion of the lung architecture [1] with histological changes including honeycombing, sparse cellular inflammation and fibroblastic foci [2]. It carries a poor prognosis, with a mean survival of 2.9 years from the time of diagnosis [3,4], there is considerable variability in outcome and prognostic indicators are required. This study investigated whether baseline plasma VEGF concentration and CANO are associated with disease progression or mortality in IPF.

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