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:
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.
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