Severe Pulmonary Arterial Hypertension Is Characterized by Increased Neutrophil Elastase and Relative Elafin Deficiency.

ABSTRACT:

Background

Preclinical evidence implicates neutrophil elastase (NE) in pulmonary arterial hypertension (PAH) pathogenesis, and the NE inhibitor elafin is under early therapeutic investigation.

Research question

Are circulating NE and elafin levels abnormal in PAH and are they associated with clinical severity?

Study design and methods

In an observational Stanford University PAH cohort (n = 249), plasma NE and elafin levels were measured in comparison with those of healthy control participants (n = 106). NE and elafin measurements were then related to PAH clinical features and relevant ancillary biomarkers. Cox regression models were fitted with cubic spline functions to associate NE and elafin levels with survival. To validate prognostic relationships, we analyzed two

SUBMITTER: Sweatt AJ 

PROVIDER: S-EPMC8546243 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature