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Pulmonary vasodilator therapy has transformed pulmonary arterial hypertension from a uniformly fatal disease to a chronically managed one. Clinicians also use these agents to treat patients with pulmonary hypertension secondary to interstitial lung disease (ILD); whether this practice is helpful or harmful is unclear.
Investigators randomized 326 patients with ILD (diffuse parenchymal disease on computed tomography) and right heart catheter–confirmed pulmonary hypertension to receive either inhaled treprostinil or placebo. Nearly three quarters of patients used supplemental oxygen, and the majority had not received pharmacologic therapy previously for ILD.
At week 16, mean 6-minute walk distance had increased by 21 m in the treprostinil group…