• Zeenat Safdar, Adaani Frost, Emilio Tameez, and Mark Entman.
• Chest. 2014 Mar 1;145(3 Suppl):535A.

Session TitleDVT/PE/Pulmonary HypertensionSESSION TYPE: Slide PresentationsPRESENTED ON: Sunday, March 23, 2014 at 12:15 PM - 01:15 PMPURPOSE: The goal of this study was to determine if biomarkers of collagen metabolism in idiopathic, hereditary and anorexigen associated pulmonary arterial hypertension (PAH) identify patients with worse disease and higher risk of death.MethodsStable idiopathic, anorexigen-associated and hereditary PAH patients were prospectively enrolled. Collagen biomarkers levels were measured: N-terminal propeptide of type III procollagen (PIIINP), C-terminal telopeptide of collagen type I (CITP), matrix metalloproteinsase 9 (MMP-9) and tissue inhibitor of metalloproteinase 1 (TIMP-1). Patients were divided into mild, moderate, and severe PAH groups. Data was compared between tertiles of each biomarker. Pearson correlation and Spearman rank coefficient analyses were performed. Data on time to death or transplantation was examined by Kaplan-Meier survival curves.ResultsCirculating levels of PIIINP, CITP, MMP9 and TIMP1 were higher in the PAH group as compared to age- and gender-matched normal controls (p<0.001 for each). PIIINP levels increased with the severity of disease (p=0.002), it was lowest in patients with mild PAH and highest in patients with severe PAH. PIIINP tertile data indicated that with increasing levels, six-minute walk distance (6MWD) and cardiac index (CI) decreased and WHO FC worsened, and resting heart rate increased. A significant correlation existed between PIIINP with worsening WHO FC (rs=0.319, p=0.008) and a negative correlation with CI and 6MWD (r=-0.304 and -0.361 respectively; p<0.05). PIIINP tertiles showed a trend towards worse outcome in patients with higher tertile (lung transplant or death) (p=0.07, log rank test).ConclusionsMarkers of collagen metabolism were associated with disease severity in PAH patients.Clinical ImplicationsOur data suggests that circulating collagen biomarkers are elevated in patients with PAH and that the state of active vascular remodeling in the pulmonary vascular bed may be represented by circulating collagen biomarkers.DisclosureZeenat Safdar: Consultant fee, speaker bureau, advisory committee, etc.: Acetelion, United Therapeutics, Gilead sciences and Bayer pharmaceuticals Adaani Frost: Consultant fee, speaker bureau, advisory committee, etc.: Gilead sciences, Bayer, United therapeutics, Acetlion, pfizer The following authors have nothing to disclose: Emilio Tameez, Mark EntmanNo Product/Research Disclosure Information.

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