title: Collagen Metabolism Biomarkers and Health Related Quality of Life in Pulmonary Arterial Hypertension.
abstract: OBJECTIVE
  The goal of this study was to investigate the association between collagen metabolism biomarkers and health related quality of life (HRQoL) in PAH patients.
  
  
  METHODS
  We prospectively enrolled 68 stable idiopathic, anorexigen-associated, and hereditary PAH subjects and 37 healthy controls. Serum samples were analyzed for N-terminal propeptide of type III procollagen (PIIINP), c-terminal telopeptide of collagen type I (CITP), matrix metalloproteinase 9 (MMP-9) and tissue inhibitor of metalloproteinase 1 (TIMP-1). The Minnesota Living with Heart Failure (MLWHF), Euro QoL-5D (EQ-5D), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and Short Form (SF-36) general health survey were administered at the time of blood draw. General linear models, as well as logistic regression models were used to assess associations between variables.
  
  
  RESULTS
  CITP, PIIINP, MMP9, and TIMP1 levels, and all HRQoL domains were significantly different between controls and PAH patients (p<0.001 for each). Interestingly, PIIINP levels were significantly associated with MLWHF physical (coef=1.63, and p=0.02), SF-36 physical (coef=-2.93, p=0.004), and EQ-5D aggregate (coef=0.34, p=0.001) scores. Several of the CAMPHOR scores strongly linearly associated with PIIINP. The odds of obtaining a walk distance ≥330 meters decrease by 38% per unit increase in PIIINP (OR=0.62; 95% CI=0.43, 0.90) and a PIIINP cutoff of 5.53 μg/L provided 81% sensitivity and 82% specificity.
  
  
  CONCLUSIONS
  PIIINP is a good predictor of disease severity, and is strongly related to HRQoL scores in PAH patients. These relationships suggest PIIINP as a promising tool for PAH clinicians to determine or confirm the level of disease severity.
authors.affiliation: Division of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine, USA.
authors.forename: Zeenat
authors.initials: Z
authors.lastname: Safdar
authors.affiliation: Division of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine, USA.
authors.forename: Emilio
authors.initials: E
authors.lastname: Tamez
authors.affiliation: Division of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine, USA.
authors.forename: Adaani
authors.initials: A
authors.lastname: Frost
authors.affiliation: Dan L Duncan Institute for Clinical and Translational Research, Baylor College of Medicine, USA.
authors.forename: Danielle
authors.initials: D
authors.lastname: Guffey
authors.affiliation: Dan L Duncan Institute for Clinical and Translational Research, Baylor College of Medicine, USA.
authors.forename: Charles G
authors.initials: CG
authors.lastname: Minard
authors.affiliation: Division of Cardiology, Baylor College of Medicine, USA.
authors.forename: Mark L
authors.initials: ML
authors.lastname: Entman