PHA Proof of Concept Research Grant Winner 2014

Daniel L. Fox, MD

Daniel L. Fox, MD

Fellow, Pulmonary Sciences & Critical Care Medicine
University of Colorado, Aurora, Colorado
Title: “SSc-PAH Risk Score: Early Identification of Scleroderma-associated PAH by RV Strain, GDF-15 & IL&r”
Term: December 1, 2014- November 30, 2015

Summary of Research Project:

The development of pulmonary arterial hypertension (PAH) occurs in 10-20% of patients with a diagnosis of scleroderma, making this population a vitally important one to screen for the development of PAH. Through the use of novel, non-invasive echocardiographic techniques targeting “right ventricular strain”- a new way to measure and quantify RV function- Dr. Fox and his team believe that patients with scleroderma-associated PAH can now be identified earlier and stratified into risk groups. Dr. Fox envisions combining RV strain analysis with emerging blood-derived biomarkers to create a composite “SSc-PAH Risk Score” that will allow clinicians to risk-stratify patients with SSc, based on their risk of having early-stage PAH. Patients at lower risk based on their RV strain and serum biomarker data could therefore be monitored clinically, while those patients with a higher risk score would proceed to definitive diagnostic testing via right heart catheterization. Further, utilizing the teams unique biobank of patients with scleroderma who later develop PAH will allow for the identification of additional biomarkers of early disease, further honing our ability to detect SSc-PAH early in its course. This new screening tool would fundamentally alter the landscape of clinical practice, offering the promise of decreasing invasive procedures, improving early detection of disease and potentially changing when and how PAH-specific therapy is initiated, potentially abrogating morbidity and mortality in this devastating disease.