Volume 20: Issue 4

INTRODUCTION

Pulmonary hypertension (PH) has long been recognized as a complication of interstitial lung disease (ILD). It contributes significantly to morbidity and mortality and thus is of key importance in prognostication and deciding the timing of referral for lung transplant. There is increasing evidence of the complexity of its pathogenesis beyond simple fibrosis and hypoxemic vasoconstriction. The pathophysiologic overlap with pulmonary arterial hypertension (PAH) has led to trials of pulmonary vasodilatory therapy in PH-ILD. While prior trials of pulmonary vasodilatory therapy in ILD have presented mixed results, a recent trial of inhaled pulmonary vasodilator therapy in this group has shown

Connective tissue diseases are a multisystem disorder that can cause impairments in quality of life, shorten life expectancy, and increase the risk of mortality at a younger age. These patients have an increased risk for the development of pulmonary hypertension through several mechanisms including pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease. This review aims to discuss the various presentations of connective tissue disease associated with pulmonary hypertension associated with interstitial lung disease, demographics, and survival. It gives an overview of accepted mechanisms of disease pathogenesis, discusses advances in diagnostics, and treatment options. Despite a deeper understanding of disease pathogenesis, treatment for this remains limited to prevention of disease progression. The identification of the primary disease driver requires careful evaluation of the disease phenotype and is a potential target for treatment and prevention of death.

Pulmonary hypertension associated with interstitial lung disease signifies worse outcomes. Given previous negative clinical trials, the use of pulmonary vasodilators in pulmonary hypertension associated with interstitial lung disease has traditionally been on a case-by-case basis; however, the recent INCREASE study has led to the first and milestone approval of inhaled treprostinil for this population. This review discusses the management of pulmonary hypertension associated with interstitial lung disease from the pulmonary vascular perspective, with an emphasis on clinical trials in this population.

Premature infants are at risk of developing bronchopulmonary dysplasia and associated pulmonary hypertension. These infants make up a complex group of patients with unique considerations regarding development of lung and vascular disease, comorbidities, and care plans. They are high risk for many complications and poor outcomes due to the severity and complexity of disease. Because of this, a comprehensive approach to care with consideration for multiple organ systems and with an interdisciplinary team of experts is the preferred approach. Here we describe in detail the major considerations in care for these infants.

Dr Edelman: Good morning. I’d like to start with discussing some general concepts, and how we approach these patients. Pulmonary hypertension is certainly common in patients with interstitial lung disease. There’s been an increasing awareness of the prevalence of interstitial lung disease accompanied by increased identification of interstitial lung disease patients with associated pulmonary hypertension.

We have a burgeoning population of patients that we are seeing and it’s important to discuss how we approach these patients in our clinics. It might be prudent to start with discussing the evaluation of these patients, and how it may be different than

Full Issue PDF

Editor’s Memo

PH-ILD: Identification, Evaluation, and Monitoring: A Diagnostic View From Both Sides

INTRODUCTION

Connective Tissue Disease, Interstitial Lung Disease, and Pulmonary Hypertension (CTD PH-ILD): A Distinct Entity and Potential Opportunity

Management of PH-ILD: Past, Present, and Future

PH Professional Network: 360-degree Care for the Bronchopulmonary Dysplasia Infant with Pulmonary Hypertension: A Comprehensive Review

PH Roundtable: Conundrums and Controversies in PH-ILD: To Treat or Not to Treat—Identifying Optimal Treatment Candidates

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease