Idiopathic pulmonary fibrosis (IPF) is a rare, chronic and life-threatening disease in which the tissues of the lungs become progressively thick and stiff.

As a result of thickening of lung tissues, brain and other organs are unable to receive optimum oxygen. There is no cure for IPF. The clinical prognosis of patients with IPF remains poor as the median survival at diagnosis is 2 to 5 years.

Importantly, pulmonary hypertension (PH) is considered as one of the principal predictors of mortality in patients with IPF. Although current treatment options are available, they don’t improve lung function, and the disease continues to progress in the majority of patients.

Therefore, there is still significant unmet medical need in IPF.