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

Article Critical Care Medicine

Inhaled Treprostinil Dosage in Pulmonary Hypertension Associated With Interstitial Lung Disease and Its Effects on Clinical Outcomes

Steven D. Nathan et al.

Summary: Higher dosages of inhaled treprostinil have been shown to have greater benefits in preventing clinical worsening and achieving clinical improvement in patients with pulmonary hypertension resulting from interstitial lung disease.

CHEST (2023)

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Article Critical Care Medicine

Efficacy of Inhaled Treprostinil on Multiple Disease Progression Events in Patients with Pulmonary Hypertension due to Parenchymal Lung Disease in the INCREASE Trial

Steven D. Nathan et al.

Summary: The study showed that patients treated with inhaled treprostinil had better outcomes in terms of 6-minute walk distance and fewer clinical worsening events. There were fewer occurrences of multiple disease progression events in the inhaled treprostinil group, indicating a lower risk of further disease progression in these patients.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Critical Care Medicine

Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Ganesh Raghu et al.

Summary: This article provides updates on the guidelines for idiopathic pulmonary fibrosis (IPF) and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. The recommendations are based on consensus and systematic reviews, aiming to provide evidence-based guidance for clinicians.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Respiratory System

The Effect of Borderline Pulmonary Hypertension on Survival in Chronic Lung Disease

Lucilla Piccari et al.

Summary: The impact of borderline pulmonary hypertension (PH) on survival in patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) remains unclear. However, in ILD, any level of PH is associated with worse survival, while in COPD, only severe PH leads to poorer outcomes.

RESPIRATION (2022)

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Article Critical Care Medicine

A Phase IIb Randomized Clinical Study of an Anti-αvβ6 Monoclonal Antibody in Idiopathic Pulmonary Fibrosis

Ganesh Raghu et al.

Summary: The efficacy and safety of BG00011, an anti-avb6 IgG1 monoclonal antibody, in the treatment of idiopathic pulmonary fibrosis (IPF) were evaluated in a phase IIb randomized controlled trial. The results showed that there was no significant difference in FVC change from baseline between patients who received BG00011 or placebo at Week 26. However, patients in the BG00011 group showed a worsening trend after Week 26 and serious adverse events occurred more frequently in BG00011 patients, including four deaths. The study suggests that further research is needed to identify new treatment strategies for IPF.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Critical Care Medicine

Impairment of the NKT-STAT1-CXCL9 Axis Contributes to Vessel Fibrosis in Pulmonary Hypertension Caused by Lung Fibrosis

Katharina Jandl et al.

Summary: This study identified a functional link between natural killer T (NKT) cell activation and vascular fibrosis in patients with pulmonary fibrosis-associated pulmonary hypertension (PF-PH). Activation of NKT cells ameliorated vascular remodeling and right ventricular systolic pressure in a mouse model of PF-PH. Furthermore, activation of the STAT1-CXCL9-CXCR3 axis by NKT cell activation reduced collagen deposition in human pulmonary arterial smooth muscle cells and precision-cut lung slices. These findings suggest a novel therapeutic strategy for targeting vascular fibrosis in interstitial lung diseases.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

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Article Critical Care Medicine

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis

Marius M. Hoeper et al.

Summary: This study provides a detailed characterization of patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH) and identifies a distinct lung phenotype characterized by low diffusion capacity for carbon monoxide (DLCO) and smoking history. These patients resemble those with pulmonary hypertension due to lung disease rather than classical IPAH. These findings have important implications for understanding the pathogenesis, diagnosis, and treatment of the disease and warrant further exploration.

LANCET RESPIRATORY MEDICINE (2022)

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Article Respiratory System

Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis

Steven D. Nathan et al.

Summary: This study aims to investigate the effectiveness of inhaled treprostinil in the treatment of IPF. The study design includes two replicate, 52-week, randomized, double-blind placebo-controlled phase 3 studies, enrolling a total of 396 subjects. The primary endpoint is the change in absolute FVC at week 52, and secondary endpoints include time to clinical worsening, time to first acute exacerbation of IPF, overall survival, and changes in questionnaire scores. Safety parameters will also be assessed, and there will be an open-label extension study for patients who complete week 52.

BMJ OPEN RESPIRATORY RESEARCH (2022)

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Article Cardiac & Cardiovascular Systems

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG)

Marc Humbert et al.

EUROPEAN HEART JOURNAL (2022)

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Review Cardiac & Cardiovascular Systems

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

Steven D. D. Nathan et al.

Summary: This paper summarizes prior studies in PH-ILD and provides suggestions and guidance for future clinical trials. Factors such as patient phenotype, inclusion criteria, clinical trial endpoints, and pharmacovigilance need to be considered in these studies.

PULMONARY CIRCULATION (2022)

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Article Respiratory System

A Phase-2 Exploratory Randomized Controlled Trial of INOpulse in Patients with Fibrotic Interstitial Lung Disease Requiring Oxygen

Christopher S. King et al.

Summary: Treatment with INOpulse improves physical activity and symptoms in patients with interstitial lung disease requiring supplemental oxygen, with good tolerability.

ANNALS OF THE AMERICAN THORACIC SOCIETY (2022)

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Article Critical Care Medicine

Efficacy and safety of sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: a double-blind, randomised, placebo-controlled, phase 2b trial

Juergen Behr et al.

Summary: The study aimed to assess the efficacy and safety of sildenafil added to pirfenidone versus placebo in patients with advanced IPF at risk of pulmonary hypertension. The results showed that sildenafil did not provide a treatment benefit compared to placebo up to 52 weeks, with no new safety signals identified. Further research is needed to determine if specific subgroups of IPF patients might benefit from sildenafil.

LANCET RESPIRATORY MEDICINE (2021)

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Article Medicine, General & Internal

Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease

Aaron Waxman et al.

Summary: In patients with pulmonary hypertension due to interstitial lung disease, inhaled treprostinil significantly improved exercise capacity and clinical outcomes compared to placebo in a 16-week trial. Adverse events such as cough, headache, dyspnea, dizziness, nausea, fatigue, and diarrhea were commonly reported.

NEW ENGLAND JOURNAL OF MEDICINE (2021)

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Article Cardiac & Cardiovascular Systems

Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study

Steven D. Nathan et al.

Summary: The study analyzed patients with pulmonary hypertension associated with idiopathic interstitial pneumonia using high-resolution computed tomography scans. It found that patients with combined pulmonary fibrosis and emphysema (CPFE) had higher mortality rates, with those having more emphysema than fibrosis facing increased risk of poor outcomes.

JOURNAL OF HEART AND LUNG TRANSPLANTATION (2021)

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Letter Respiratory System

Pulmonary vascular resistance predicts mortality in patients with pulmonary hypertension associated with interstitial lung disease: results from the COMPERA registry

Karen M. Olsson et al.

Summary: Pulmonary vascular resistance is a stronger prognostic indicator than mean pulmonary arterial pressure in patients with pulmonary hypertension associated with interstitial lung disease.

EUROPEAN RESPIRATORY JOURNAL (2021)

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Article Critical Care Medicine

Inhaled treprostinil and forced vital capacity in patients with interstitial lung disease and associated pulmonary hypertension: a post-hoc analysis of the INCREASE study

Steven D. Nathan et al.

Summary: The INCREASE trial demonstrated that inhaled treprostinil improved FVC in patients with ILD and associated pulmonary hypertension, with more significant effects observed in specific subgroups, suggesting its potential as a therapeutic option for pulmonary fibrosis.

LANCET RESPIRATORY MEDICINE (2021)

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Article Physiology

Lung Pericytes in Pulmonary Vascular Physiology and Pathophysiology

Ke Yuan et al.

Summary: Pericytes are mural cells derived from mesenchyme located within the basement membrane of pulmonary and systemic capillaries, playing important roles in vascular homeostasis, angiogenesis, and pathology. Dysregulation of pericyte communication and phenotype switching are common features of lung disorders associated with vascular remodeling, inflammation, and fibrosis, indicating their potential involvement in the pathogenesis of these diseases.

COMPREHENSIVE PHYSIOLOGY (2021)

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Article Cardiac & Cardiovascular Systems

Impact of the new definition for pulmonary hypertension in patients with lung disease: an analysis of the United Network for Organ Sharing database

Steven D. Nathan et al.

Summary: The recent change in the definition of pulmonary hypertension has significant implications on the epidemiology and outcomes of patients with lung diseases. The study found that under the new definition, there is a higher percentage of patients with pulmonary hypertension in both chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis. Patients with pulmonary hypertension, regardless of the definition applied, had shorter transplant-free waitlist survival. Additionally, the new definition of pre-capillary pulmonary hypertension may provide slightly better discernment of outcomes in idiopathic pulmonary fibrosis patients compared to the old definition.

PULMONARY CIRCULATION (2021)

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Article Critical Care Medicine

A Randomized, Double-Blind, Placebo-Controlled Study of Pulsed, Inhaled Nitric Oxide in Subjects at Risk of Pulmonary Hypertension Associated With Pulmonary Fibrosis

Steven D. Nathan et al.

CHEST (2020)

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Article Medicine, Research & Experimental

A win ratio approach to the re-analysis of Multiple Risk Factor Intervention Trial

Ales Kotalik et al.

CLINICAL TRIALS (2019)

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Article Medicine, General & Internal

Nintedanib in Progressive Fibrosing Interstitial Lung Diseases

K. R. Flaherty et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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Article Critical Care Medicine

Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study

Steven D. Nathan et al.

LANCET RESPIRATORY MEDICINE (2019)

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Article Respiratory System

Haemodynamic definitions and updated clinical classification of pulmonary hypertension

Gerald Simonneau et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

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Article Respiratory System

Pulmonary hypertension in chronic lung disease and hypoxia

Steven D. Nathan et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

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Article Medicine, General & Internal

Nintedanib plus Sildenafil in Patients with Idiopathic Pulmonary Fibrosis

Martin Kolb et al.

NEW ENGLAND JOURNAL OF MEDICINE (2018)

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Article Cardiac & Cardiovascular Systems

Pulmonary hypertension in chronic lung diseases: comparison to other pulmonary hypertension groups

Nader Chebib et al.

PULMONARY CIRCULATION (2018)

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Article Respiratory System

Progression of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis with mild to moderate restriction

Ryo Teramachi et al.

RESPIROLOGY (2017)

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Article Critical Care Medicine

Efficacy of simtuzumab versus placebo in patients with idiopathic pulmonary fibrosis: a randomised, double-blind, controlled, phase 2 trial

Ganesh Raghu et al.

LANCET RESPIRATORY MEDICINE (2017)

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Article Biochemistry & Molecular Biology

Targeting of the pulmonary capillary vascular niche promotes lung alveolar repair and ameliorates fibrosis

Zhongwei Cao et al.

NATURE MEDICINE (2016)

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Article Respiratory System

Severe pulmonary hypertension in lung disease: phenotypes and response to treatment

Melanie J. Brewis et al.

EUROPEAN RESPIRATORY JOURNAL (2015)

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Article Multidisciplinary Sciences

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

Marius M. Hoeper et al.

PLOS ONE (2015)

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Article Critical Care Medicine

Bosentan in Pulmonary Hypertension Associated with Fibrotic Idiopathic Interstitial Pneumonia

Tamera J. Corte et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)

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Article Respiratory System

Changes in right heart haemodynamics and echocardiographic function in an advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis

Rajeev Saggar et al.

THORAX (2014)

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Article Medicine, General & Internal

Treatment of Idiopathic Pulmonary Fibrosis With Ambrisentan A Parallel, Randomized Trial

Ganesh Raghu et al.

ANNALS OF INTERNAL MEDICINE (2013)

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Article Critical Care Medicine

Sildenafil Preserves Exercise Capacity in Patients With Idiopathic Pulmonary Fibrosis and Right-sided Ventricular Dysfunction

MeiLan K. Han et al.

CHEST (2013)

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Article Respiratory System

Riociguat for interstitial lung disease and pulmonary hypertension: a pilot trial

Marius M. Hoeper et al.

EUROPEAN RESPIRATORY JOURNAL (2013)

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Article Respiratory System

Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial

Ganesh Raghu et al.

EUROPEAN RESPIRATORY JOURNAL (2013)

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Article Cardiac & Cardiovascular Systems

Current practice for determining pulmonary capillary wedge pressure predisposes to serious errors in the classification of patients with pulmonary hypertension

John J. Ryan et al.

AMERICAN HEART JOURNAL (2012)

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Article Cardiac & Cardiovascular Systems

The win ratio: a new approach to the analysis of composite endpoints in clinical trials based on clinical priorities

Stuart J. Pocock et al.

EUROPEAN HEART JOURNAL (2012)

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Article Critical Care Medicine

BUILD-3: A Randomized, Controlled Trial of Bosentan in Idiopathic Pulmonary Fibrosis

Talmadge E. King et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2011)

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Article Respiratory System

Sildenafil Therapy and Exercise Tolerance in Idiopathic Pulmonary Fibrosis

Robert M. Jackson et al.

LUNG (2010)

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Article Medicine, General & Internal

A Controlled Trial of Sildenafil in Advanced Idiopathic Pulmonary Fibrosis

David A. Zisman et al.

NEW ENGLAND JOURNAL OF MEDICINE (2010)

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Article Respiratory System

The use of sildenafil to treat pulmonary hypertension associated with interstitial lung disease

Tamera J. Corte et al.

RESPIROLOGY (2010)

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Article Critical Care Medicine

Misclassification of Pulmonary Hypertension Due to Reliance on Pulmonary Capillary Wedge Pressure Rather Than Left Ventricular End-Diastolic Pressure

Scott D. Halpern et al.

CHEST (2009)

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Article Respiratory System

Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis

Steven D. Nathan et al.

RESPIRATION (2008)

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Article Cardiac & Cardiovascular Systems

Vaso-active therapy can improve 6-min walk distance in patients with pulmonary hypertension and fibrotic interstitial lung disease

Omar A. Minai et al.

RESPIRATORY MEDICINE (2008)

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Article Critical Care Medicine

Idiopathic pulmonary fibrosis and pulmonary hypertension - Connecting the dots

Steven D. Nathan et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2007)

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Article Critical Care Medicine

Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis

Steven D. Nathan et al.

CHEST (2007)

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Article Pathology

Pulmonary vascular lesions in end-stage idiopathic pulmonary fibrosis: histopathologic study on lung explant specimens and correlations with pulmonary hemodynamics

Magali Colombat et al.

HUMAN PATHOLOGY (2007)

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Article Critical Care Medicine

Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis

CJ Lettieri et al.

CHEST (2006)

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Article Pathology

The role of microvascular injury in the evolution of idiopathic pulmonary fibrosis

CM Magro et al.

AMERICAN JOURNAL OF CLINICAL PATHOLOGY (2003)

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Article Medicine, General & Internal

Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial

HA Ghofrani et al.

LANCET (2002)

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

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