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

Abstract

Purpose of Review: This review delineates current diagnostic and management strategies for pulmonary hypertension due to interstitial lung disease (PH‐ILD). Recent Findings: 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. Summary: 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.