For Providers & Researchers

[PLACEHOLDER—wording to be provided by study team] ATHENA is “A Phase II, Randomized, Double-Blind, Safety and Efficacy Study of Tiprelestat Versus Placebo when Added to Standard of Care for the Treatment of Pulmonary Arterial Hypertension (PAH).”

Prior research has demonstrated that occlusive vascular pathology in PAH is in part related to vascular elastases which breakdown the elastic laminae in walls of pulmonary arteries, resulting in vascular smooth muscle cell and adventitial fibroblast proliferation and migration, as well as promotion of local and systemic inflammatory response.

Elafin is an endogenous elastase inhibitor which is deficient relative to excess levels of human neutrophil elastase (HNE) in PAH patients. We have shown that human recombinant elafin (known as tiprelestat) attenuates development of pulmonary vascular neointimal lesions, reverses PAH in rodent models, regresses PAH lesions in human lung tissue in culture, and promotes activation of BMPR2. Therefore, we hypothesize that tiprelestat is safe and effective in treating PAH. Please visit clinicaltrials.gov for more information.

ATHENA is funded by the National Institutes of Health (NIH) / National Heart, Lung, Blood Institute (NHLBI). Please visit NIH RePORTER for more information.

If you are interested in more information on this study, please contact the ATHENA study Project Managers at ATHENA-PM@umich.edu.