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For the treatment of pulmonary hypertension associated with interstitial lung disease (PH-ILD; WHO Group 3) to improve exercise ability.

FOR US HEALTHCARE PROFESSIONALS ONLY

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PATHOPHYSIOLOGY

ILD can cause changes to the lung microvasculature2-4

PH-ILD pathophysiology is multifactorial. Pulmonary vascular changes that occur due to ILD may include2-4:

The pathophysiology of interstitial lung disease (ILD) involves changes to the lungs such as abnormal microvasculature, hypoxia-induced vasoconstriction, and pulmonary vascular remodeling. These alterations increase pulmonary vascular resistance and can contribute to the development of pulmonary hypertension.

Abnormal microvasculature due to fibrotic destruction and/or disrupted angiogenesis

AND

vasoconstriction and vascular remodeling throughout the lungs due to repeated or sustained hypoxic conditions and/or fibrosis

AND

additional vasculopathy associated with molecular and genetic abnormalities present only in patients with ILD who have PH

Even less severe PH in patients with ILD is clinically significant. Slightly elevated pulmonary pressures add considerable circulatory strain to patients who already have ventilation defects.5,6

ILD=interstitial lung disease; PH=pulmonary hypertension.