Objectives: 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), EQ-5D (EQ-5D),
Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and Short Form
(SF-36) general health survey were administere
d 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 the
best 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.Read more......
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