Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative—Group 3 pulmonary hypertension

Abstract

Pulmonary hypertension (PH) has been linked to worse outcomes in chronic lung diseases. The presence of PH in the setting of underlying Interstitial Lung Disease (ILD) is strongly associated with decreased exercise and functional capacity, an increased risk of hospitalizations and death. Examining the scope of this issue and its impact on patients is the first step in trying to define a roadmap to facilitate and encourage future research in this area. The aim of our working group is to strengthen the communities understanding of PH due to lung diseases and to improve the care and quality of life of affected patients. This introductory statement provides a broad overview and lays the foundation for further in‐depth papers on specific topics pertaining to PH‐ILD.

Pulmonary hypertension in interstitial lung disease: Clinical trial design and endpoints: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative—Group 3 Pulmonary Hypertension

“…The lack of mandated RHC-confirmed PH could have played a role in the negative results of some of these clinical trials, especially in the aforementioned studies of sildenafil, although there have been a number of negative studies where RHC was mandatory to confirm the diagnosis of PH. 1,16 The largest randomized controlled study targeting PH-ILD, the INCREASE study, included 346 patients equally randomized between inhaled treprostinil and placebo. 17 The study met its primary endpoint of change in the 6-minute walk distance at 16 weeks.…”

“…However, there were no differences in reported serious adverse events or death between both treatment groups. The lack of mandated RHC‐confirmed PH could have played a role in the negative results of some of these clinical trials, especially in the aforementioned studies of sildenafil, although there have been a number of negative studies where RHC was mandatory to confirm the diagnosis of PH 1,16 …”

“…The initial article of this series from PVRI's Group 3 Pulmonary Hypertension Workstream examined the scope of this issue and its impact on patients as the first step to facilitate and encourage future research in this area. 1 The advent of therapeutics for Group 1 pulmonary arterial hypertension (PAH) has raised interest in studying and using these drugs in Group 3 pulmonary hypertension (PH), especially the ILDs. This paper is designed to provide an overview of prior clinical trials, including lessons from these studies that can help provide a roadmap for future clinical drug studies targeting the pulmonary vasculature or pulmonary hypertension in patients with ILD (PH‐ILD).…”

“…Pulmonary hypertension (PH) complicates the course of many patients suffering from interstitial lung disease (ILD). The initial article of this series from PVRI's Group 3 Pulmonary Hypertension Workstream examined the scope of this issue and its impact on patients as the first step to facilitate and encourage future research in this area 1 . The advent of therapeutics for Group 1 pulmonary arterial hypertension (PAH) has raised interest in studying and using these drugs in Group 3 pulmonary hypertension (PH), especially the ILDs.…”

Die Lunge: Ausgangspunkt vieler Erkrankungen

“…Das gleichzeitige Vorliegen einer SSc-assoziierten ILD und pulmonalen Druckerhöhung stellt in der täglichen Praxis eine Herausforderung dar. Diese Patienten leiden oftmals an deutlich stärkeren Symptomen, die sich aus einer Kombination von Husten, Dyspnoe, Müdigkeit, Schwindel und Ödemen zusammensetzen und mit einem großen Leidensdruck einhergehen [ 18 ].…”