Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare, chronic and life-threatening disease characterized by an abnormal and progressive increase in blood pressure in the small pulmonary arteries leading to hypertrophy of the right ventricle of the heart and ultimately to right heart failure and death. 

This increased blood pressure is caused by inflammation, vascular smooth muscle cell proliferation and subsequent narrowing of the small pulmonary arteries which are the blood vessels that carry blood from the right side of the heart through the lungs. NLRP3 Inflammasome activation has been identified as an important driver of continued arterial inflammation in PAH and of hypertrophy of the right ventricle of the heart.

There is currently no cure for PAH. Available therapies are agents that focus on improving the vasoconstriction-vasodilation balance without addressing the underlying inflammatory cause of the disease. As a result, five-year median survival remains relatively low despite treatment.

PAH has an estimated prevalence in Europe of 15–60 cases per million.