Pulmonary Hypertension in Interstitial Lung Disease: Management Options to Move Beyond Supportive Care.

Abstract

This review delineates current diagnostic and management strategies for pulmonary hypertension due to interstitial lung disease (PH-ILD). The INCREASE trial, a phase III multicenter, randomized, placebo-controlled trial demonstrated both improved 6-min walk distance and decreased disease progression with inhaled treprostinil. This pivotal trial led to inhaled treprostinil becoming the first FDA approved medication for treatment of PH-ILD. The availability of this treatment has generated subsequent recommendations for the screening for PH in patients with ILD. As a result, it is becoming increasingly important for clinicians to gain awareness and familiarity with the evolving management options for PH-ILD. The management of PH-ILD has its roots in goal-directed treatment of the underlying lung disease. However, recent medication advances and ongoing clinical studies are opening opportunities for more disease-specific treatment.