Pharmacological management of connective tissue disease-associated pulmonary arterial hypertension

Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension

(2023) Marius M. Hoeper et al.   NEW ENGLAND JOURNAL OF MEDICINE

Upfront riociguat and ambrisentan combination therapy for newly diagnosed pulmonary arterial hypertension: A prospective open-label trial

(2022) Jason Weatherald et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Improved Survival for Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension: The Johns Hopkins Registry

(2022) Hussein J Hassan et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

(2022) Marc Humbert et al.   EUROPEAN HEART JOURNAL

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

(2022) Marc Humbert et al.   EUROPEAN RESPIRATORY JOURNAL

Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease

(2021) Aaron Waxman et al.   NEW ENGLAND JOURNAL OF MEDICINE

Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis Associated Pulmonary Arterial Hypertension: A Multi-center, Double-blind, Randomized, Placebo-controlled Trial

(2021) Roham T Zamanian et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Long‐Term Outcomes in Patients with Connective Tissue Disease‐Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta‐Analyses of Randomized, Controlled Trials and Observational Registries

(2021) Dinesh Khanna et al.   Arthritis & Rheumatology

Sotatercept for the Treatment of Pulmonary Arterial Hypertension

(2021) Marc Humbert et al.   NEW ENGLAND JOURNAL OF MEDICINE

Nailfold Capillaroscopy in Systemic Sclerosis Patients with and without Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

(2021) Ioanna Minopoulou et al.   Journal of Clinical Medicine

Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation

(2021) Lorriana E. Leard et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Three- Versus Two-Drug Therapy for Patients With Newly Diagnosed Pulmonary Arterial Hypertension

(2021) Kelly M. Chin et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension

(2021) Frédéric Perros et al.   European Respiratory Review

Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension

(2020) Olivier Sitbon et al.   EUROPEAN RESPIRATORY JOURNAL

Comparison of V/Q SPECT and CT Angiography for the Diagnosis of Chronic Thromboembolic Pulmonary Hypertension

(2020) Meng Wang et al.   RADIOLOGY

Hemodynamic response to treatment and outcome in pulmonary hypertension associated with interstitial lung disease versus pulmonary arterial hypertension in systemic sclerosis

(2020) Louis Chauvelot et al.   Arthritis & Rheumatology

Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial

(2020) Ekkehard Grünig et al.   EUROPEAN HEART JOURNAL

Pulmonary hypertension in connective tissue diseases, new evidence and challenges

(2020) Madelon C. Vonk et al.   EUROPEAN JOURNAL OF CLINICAL INVESTIGATION

ERS Statement on Chronic Thromboembolic Pulmonary Hypertension

(2020) Marion Delcroix et al.   EUROPEAN RESPIRATORY JOURNAL

Can anticoagulants improve the survival rate for patients with idiopathic pulmonary arterial hypertension? A systematic review and meta-analysis

(2020) Peijie Wang et al.   THROMBOSIS RESEARCH

Pulmonary Hypertension Associated with Connective Tissue Disease

(2019) Hossam Fayed et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

Validation of the REVEAL Prognostic Equation and Risk Score Calculator in Incident Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension

(2019) Christopher J. Mullin et al.   Arthritis & Rheumatology

Screening for pulmonary arterial hypertension in systemic sclerosis

(2019) Jason Weatherald et al.   European Respiratory Review

Combination Therapy with Oral Treprostinil for Pulmonary Arterial Hypertension: A Double-Blind, Placebo-Controlled Study

(2019) R. James White et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Results of an Expert Consensus Survey on the Treatment of Pulmonary Arterial Hypertension With Oral Prostacyclin Pathway Agents

(2019) Vallerie V. McLaughlin et al.   CHEST

The practical management of fluid retention in adults with right heart failure due to pulmonary arterial hypertension

(2019) Simone Stickel et al.   EUROPEAN HEART JOURNAL SUPPLEMENTS

Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus

(2018) Eric Hachulla et al.   CHEST

Association between Rheumatoid Arthritis and Pulmonary Hypertension: Data from the French Pulmonary Hypertension Registry

(2018) David Montani et al.   RESPIRATION

2013 ACR/EULAR systemic sclerosis classification criteria in patients with associated pulmonary arterial hypertension

(2018) Beatriz E. Joven et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

Risk assessment in scleroderma patients with newly diagnosed pulmonary arterial hypertension: application of the ESC/ERS risk prediction model

(2018) Valentina Mercurio et al.   EUROPEAN RESPIRATORY JOURNAL

Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives

(2018) Marc Humbert et al.   EUROPEAN RESPIRATORY JOURNAL

A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension

(2017) David Kylhammar et al.   EUROPEAN HEART JOURNAL

Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension

(2017) Sean Gaine et al.   EUROPEAN RESPIRATORY JOURNAL

An official European Respiratory Society statement: pulmonary haemodynamics during exercise

(2017) Gabor Kovacs et al.   EUROPEAN RESPIRATORY JOURNAL

Tadalafil in idiopathic or heritable pulmonary arterial hypertension (PAH) compared to PAH associated with connective tissue disease

(2017) Nazzareno Galiè et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Survival and outcomes after lung transplantation for non-scleroderma connective tissue–related interstitial lung disease

(2017) Andrew M. Courtwright et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Epidemiology and treatment of pulmonary arterial hypertension

(2017) Edmund M. T. Lau et al.   Nature Reviews Cardiology

Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2

(2016) Marc Humbert et al.   ANNALS OF THE RHEUMATIC DISEASES

Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial

(2016) John Gerry Coghlan et al.   ANNALS OF THE RHEUMATIC DISEASES

Connective tissue disease-related pulmonary arterial hypertension

(2016) Vivek Thakkar et al.   BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY

Initial dual oral combination therapy in pulmonary arterial hypertension

(2016) Olivier Sitbon et al.   EUROPEAN RESPIRATORY JOURNAL

Ambrisentan response in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) – A subgroup analysis of the ARIES-E clinical trial

(2016) Aryeh Fischer et al.   RESPIRATORY MEDICINE

Ambrisentan and Tadalafil Up-front Combination Therapy in Scleroderma-associated Pulmonary Arterial Hypertension

(2015) Paul M. Hassoun et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL)CLINICAL PERSPECTIVE

(2015) Ioana R. Preston et al.   CIRCULATION

Atrial Septostomy: A Contemporary Review

(2015) Hayan Al Maluli et al.   CLINICAL CARDIOLOGY

Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights

(2015) Fabio Dardi et al.   EUROPEAN RESPIRATORY JOURNAL

Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Prevalence and Predictors

(2015) Gregorio Miguel Pérez-Peñate et al.   JOURNAL OF RHEUMATOLOGY

Selexipag for the Treatment of Pulmonary Arterial Hypertension

(2015) Olivier Sitbon et al.   NEW ENGLAND JOURNAL OF MEDICINE

Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension

(2015) Nazzareno Galiè et al.   NEW ENGLAND JOURNAL OF MEDICINE

Adverse Events in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

(2015) Rennie L. Rhee et al.   Arthritis & Rheumatology

Clinical Diagnosis of Pulmonary Hypertension

(2014) Jonathan D. Rich et al.   CIRCULATION

Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients

(2014) Y.-J. Hao et al.   EUROPEAN RESPIRATORY JOURNAL

A meta-analysis of the risk of venous thromboembolism in inflammatory rheumatic diseases

(2014) Jason J Lee et al.   ARTHRITIS RESEARCH & THERAPY

Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study

(2013) J Gerry Coghlan et al.   ANNALS OF THE RHEUMATIC DISEASES

Anticoagulation and Survival in Pulmonary Arterial Hypertension

(2013) Karen M. Olsson et al.   CIRCULATION

Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses

(2013) Pia Trip et al.   EUROPEAN RESPIRATORY JOURNAL

Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension

(2013) Tomás Pulido et al.   NEW ENGLAND JOURNAL OF MEDICINE

Riociguat for the Treatment of Chronic Thromboembolic Pulmonary Hypertension

(2013) Hossein-Ardeschir Ghofrani et al.   NEW ENGLAND JOURNAL OF MEDICINE

Riociguat for the Treatment of Pulmonary Arterial Hypertension

(2013) Hossein-Ardeschir Ghofrani et al.   NEW ENGLAND JOURNAL OF MEDICINE

Pulmonary Arterial Hypertension Related to Connective Tissue Disease

(2013) Saman Ahmed et al.   RHEUMATIC DISEASE CLINICS OF NORTH AMERICA

The inclusion of N-terminal pro-brain natriuretic peptide in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension: a cohort study

(2013) Vivek Thakkar et al.   ARTHRITIS RESEARCH & THERAPY

Pulmonary hypertension in systemic lupus erythematosus: prevalence, predictors and diagnostic strategy

(2012) Guillermo Ruiz-Irastorza et al.   AUTOIMMUNITY REVIEWS

Pulmonary hypertension associated with rheumatic diseases: baseline characteristics from the Korean registry

(2012) Chan Hong Jeon et al.   International Journal of Rheumatic Diseases

Pulmonary Hypertension: How the Radiologist Can Help

(2012) Elena Peña et al.   RADIOGRAPHICS

Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: a single-centre cohort

(2012) Y. Shirai et al.   RHEUMATOLOGY

Screening for pulmonary arterial hypertension in patients with systemic sclerosis: Clinical characteristics at diagnosis and long-term survival

(2011) Marc Humbert et al.   ARTHRITIS AND RHEUMATISM

Long-term Treatment With Sildenafil Citrate in Pulmonary Arterial Hypertension

(2011) Lewis J. Rubin et al.   CHEST

Characterization of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension From REVEAL

(2010) Lorinda Chung et al.   CHEST

Left atrial volume and N-terminal pro-B type natriuretic peptide are associated with elevated pulmonary artery pressure in patients with systemic sclerosis

(2010) Theodoros Dimitroulas et al.   CLINICAL RHEUMATOLOGY

Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension

(2010) D. Montani et al.   EUROPEAN HEART JOURNAL

Addition of Inhaled Treprostinil to Oral Therapy for Pulmonary Arterial Hypertension

(2010) Vallerie V. McLaughlin et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France

(2009) Eric Hachulla et al.   ARTHRITIS AND RHEUMATISM

Tadalafil Therapy for Pulmonary Arterial Hypertension

(2009) Nazzareno Galiè et al.   CIRCULATION

Long-term outcome with intravenous iloprost in pulmonary arterial hypertension

(2009) M. M. Hoeper et al.   EUROPEAN RESPIRATORY JOURNAL

Longterm Survival Among Patients with Scleroderma-associated Pulmonary Arterial Hypertension Treated with Intravenous Epoprostenol

(2009) D. B. BADESCH et al.   JOURNAL OF RHEUMATOLOGY

Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil

(2009) D. Launay et al.   RHEUMATOLOGY

Connective Tissue Disease–associated Pulmonary Arterial Hypertension in the Modern Treatment Era

(2008) Robin Condliffe et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Immunosuppressive therapy in lupus- and mixed connective tissue disease–associated pulmonary arterial hypertension: A retrospective analysis of twenty-three cases

(2008) Xavier Jais et al.   ARTHRITIS AND RHEUMATISM

Ambrisentan for the Treatment of Pulmonary Arterial Hypertension

(2008) Nazzareno Galiè et al.   CIRCULATION