Progress in the Treatment of Pulmonary Hypertension Associated with Interstitial Lung Disease

Pulmonary hypertension, frequently complicating the course of patients with fibrotic interstitial lung disease, is associated with significantly increased morbidity and mortality. The availability of multiple medications to treat pulmonary arterial hypertension has resulted in these agents being used beyond their original indication, including in patients with interstitial lung disease. Whether pulmonary hypertension in the context of interstitial lung disease is an adaptive response not to be treated or a maladaptive phenomenon amenable to therapy has been uncertain. Although some studies have suggested a benefit of treatment, there have been others demonstrating harm. This concise clinical review provides an overview of prior studies and the issues that have plagued drug development for a patient population in dire need of treatment options. More recently, there has been a paradigm shift, with the largest study to date demonstrating benefit, resulting in the first approved therapy in the United States for patients with interstitial lung disease complicated by pulmonary hypertension. A pragmatic management algorithm in the context of changing definitions, comorbid contributors, and an available treatment option is provided, as are considerations for future clinical trials.

Automated summary

Pulmonary hypertension, a common complication of fibrotic interstitial lung disease, is associated with increased morbidity and mortality. The effectiveness of treatment for pulmonary hypertension in the context of interstitial lung disease has been uncertain, with some studies suggesting benefit and others demonstrating harm. However, recent research has shown the potential benefits of treatment, leading to the approval of the first therapy for patients with interstitial lung disease complicated by pulmonary hypertension in the United States. This review also provides a management algorithm and considerations for future clinical trials.