Pulmonary hypertension in interstitial lung disease

Abstract

Purpose of review: Exercise impairment and pulmonary hypertension are common features of interstitial lung disease. Antifibrotic therapies for interstitial lung disease remain unproved; therefore, some interest has been focused on treating the pulmonary vascular impairment these diseases.

Recent findings: Patients with pulmonary hypertension secondary to idiopathic pulmonary fibrosis may have normal resting pulmonary artery pressure, but it often rises with exercise. Exercise impairment in idiopathic pulmonary fibrosis has a strong correlation with the degree of pulmonary artery pressure elevation, and hypoxemia, a hallmark of pulmonary hypertension, strongly correlates with survival.

Summary: Small case series have shown that some patients improve on receiving therapy for pulmonary hypertension secondary to interstitial lung disease. These findings suggest that larger treatment trials for medications targeting pulmonary hypertension in interstitial lung disease are warranted.