Major lung complications of systemic sclerosis

Sirtuin1 Protects against Systemic Sclerosis–related Pulmonary Fibrosis by Decreasing Proinflammatory and Profibrotic Processes

(2018) Haiyan Chu et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Therapeutic interleukin-6 blockade reverses transforming growth factor-beta pathway activation in dermal fibroblasts: insights from the faSScinate clinical trial in systemic sclerosis

(2018) Christopher P Denton et al.   ANNALS OF THE RHEUMATIC DISEASES

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

(2018) Wanlong Wu et al.   ANNALS OF THE RHEUMATIC DISEASES

Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma

(2018) Keith M. Sullivan et al.   NEW ENGLAND JOURNAL OF MEDICINE

Radiological pleuroparenchymal fibroelastosis associated to limited cutaneous systemic sclerosis: a case report

(2018) D. Hassoun et al.   BMC Pulmonary Medicine

Association of Defective Regulation of Autoreactive Interleukin-6-Producing Transitional B Lymphocytes With Disease in Patients With Systemic Sclerosis

(2018) Taher E. Taher et al.   Arthritis & Rheumatology

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

(2017) Sean Gaine et al.   EUROPEAN RESPIRATORY JOURNAL

Monocyte-derived alveolar macrophages drive lung fibrosis and persist in the lung over the life span

(2017) Alexander V. Misharin et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Pulmonary arterial hypertension - progress in understanding the disease and prioritizing strategies for drug development

(2017) P. Ghataorhe et al.   JOURNAL OF INTERNAL MEDICINE

Early-Life Antibiotic Exposure Causes Intestinal Dysbiosis and Exacerbates Skin and Lung Pathology in Experimental Systemic Sclerosis

(2017) Heena Mehta et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Pomalidomide in Patients with Interstitial Lung Disease due to Systemic Sclerosis: A Phase II, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study

(2017) Vivien M. Hsu et al.   JOURNAL OF RHEUMATOLOGY

Scleroderma Lung Involvement, Autoantibodies, and Outcome Prediction: The Confounding Effect of Time

(2017) SVETLANA I. NIHTYANOVA et al.   JOURNAL OF RHEUMATOLOGY

An MIF Promoter Polymorphism Is Associated with Susceptibility to Pulmonary Arterial Hypertension in Diffuse Cutaneous Systemic Sclerosis

(2017) Lara Bossini-Castillo et al.   JOURNAL OF RHEUMATOLOGY

Systemic sclerosis

(2017) Christopher P Denton et al.   LANCET

Severity and features of frailty in systemic sclerosis-associated interstitial lung disease

(2017) Sabina A. Guler et al.   RESPIRATORY MEDICINE

Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort

(2017) Matthew R. Lammi et al.   RESPIROLOGY

Prognostic significance of computed tomography criteria for pulmonary veno-occlusive disease in systemic sclerosis-pulmonary arterial hypertension

(2017) Michelle J. Connolly et al.   RHEUMATOLOGY

Determinants of mortality in systemic sclerosis: a focused review

(2017) Dilli Ram Poudel et al.   RHEUMATOLOGY INTERNATIONAL

The status of pulmonary fibrosis in systemic sclerosis is associated with IRF5, STAT4, IRAK1, and CTGF polymorphisms

(2017) Wenjie Zhao et al.   RHEUMATOLOGY INTERNATIONAL

Changes in gene expression profiles in patients with pulmonary arterial hypertension associated with scleroderma treated with tadalafil

(2017) Fei-Ying Cheong et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

Rituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial

(2017) Peter Saunders et al.   Trials

A novel multi-network approach reveals tissue-specific cellular modulators of fibrosis in systemic sclerosis

(2017) Jaclyn N. Taroni et al.   Genome Medicine

Mycophenolate Mofetil Versus Placebo for Systemic Sclerosis-Related Interstitial Lung Disease: An Analysis of Scleroderma Lung Studies I and II

(2017) Elizabeth R. Volkmann et al.   Arthritis & Rheumatology

CCL2 in the Circulation Predicts Long-Term Progression of Interstitial Lung Disease in Patients With Early Systemic Sclerosis: Data From Two Independent Cohorts

(2017) Minghua Wu et al.   Arthritis & Rheumatology

Short-Term Pulmonary Function Trends Are Predictive of Mortality in Interstitial Lung Disease Associated With Systemic Sclerosis

(2017) Nicole S. Goh et al.   Arthritis & Rheumatology

Brief Report: Association of Elevated Adipsin Levels With Pulmonary Arterial Hypertension in Systemic Sclerosis

(2017) Benjamin D. Korman et al.   Arthritis & Rheumatology

Role of Stromelysin 2 (Matrix Metalloproteinase 10) as a Novel Mediator of Vascular Remodeling Underlying Pulmonary Hypertension Associated With Systemic Sclerosis

(2017) Jérôme Avouac et al.   Arthritis & Rheumatology

Early Mortality in a Multinational Systemic Sclerosis Inception Cohort

(2017) Yanjie Hao et al.   Arthritis & Rheumatology

Novel lung imaging biomarkers and skin gene expression subsetting in dasatinib treatment of systemic sclerosis-associated interstitial lung disease

(2017) Viktor Martyanov et al.   PLoS One

Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis

(2017) Johannes P. Schwaiger et al.   Scientific Reports

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

Update of EULAR recommendations for the treatment of systemic sclerosis

(2016) Otylia Kowal-Bielecka et al.   ANNALS OF THE RHEUMATIC DISEASES

Interstitial lung disease increases mortality in systemic sclerosis patients with pulmonary arterial hypertension without affecting hemodynamics and exercise capacity

(2016) M. Michelfelder et al.   CLINICAL RHEUMATOLOGY

An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Disease: the LOTUSS Trial

(2016) D. Khanna et al.   JOURNAL OF RHEUMATOLOGY

Pre-Capillary, Combined, and Post-Capillary Pulmonary Hypertension

(2016) Christian F. Opitz et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial

(2016) Dinesh Khanna et al.   LANCET

Computer-Aided Tomographic Analysis of Interstitial Lung Disease (ILD) in Patients with Systemic Sclerosis (SSc). Correlation with Pulmonary Physiologic Tests and Patient-Centred Measures of Perceived Dyspnea and Functional Disability

(2016) Fausto Salaffi et al.   PLoS One

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

BSR and BHPR guideline for the treatment of systemic sclerosis

(2016) Christopher P. Denton et al.   RHEUMATOLOGY

Esophageal dilatation and interstitial lung disease in systemic sclerosis: A cross-sectional study

(2016) Carrie Richardson et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

Derivation and External Validation of a Prediction Rule for Five-Year Mortality in Patients With Early Diffuse Cutaneous Systemic Sclerosis

(2016) Robyn T. Domsic et al.   Arthritis & Rheumatology

Characteristics and Survival of Anti-U1 RNP Antibody-Positive Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

(2016) Vincent Sobanski et al.   Arthritis & Rheumatology

Brief Report: Anti-Eukaryotic Initiation Factor 2B Autoantibodies Are Associated With Interstitial Lung Disease in Patients With Systemic Sclerosis

(2016) Zoe E. Betteridge et al.   Arthritis & Rheumatology

Combined Pulmonary Fibrosis and Emphysema in Scleroderma-Related Lung Disease Has a Major Confounding Effect on Lung Physiology and Screening for Pulmonary Hypertension

(2016) K. M. Antoniou et al.   Arthritis & Rheumatology

The American College of Rheumatology Provisional Composite Response Index for Clinical Trials in Early Diffuse Cutaneous Systemic Sclerosis

(2016) Dinesh Khanna et al.   Arthritis & Rheumatology

Endothelial to Mesenchymal Transition Contributes to Endothelial Dysfunction in Pulmonary Arterial Hypertension

(2015) Robert B. Good et al.   AMERICAN JOURNAL OF PATHOLOGY

Mechanosignaling through YAP and TAZ drives fibroblast activation and fibrosis

(2015) Fei Liu et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Impaired Bone Morphogenetic Protein Receptor II Signaling in a Transforming Growth Factor-β–Dependent Mouse Model of Pulmonary Hypertension and in Systemic Sclerosis

(2015) Adrian J. Gilbane et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE 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

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

(2015) Nazzareno Galiè et al.   EUROPEAN RESPIRATORY JOURNAL

Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension

(2015) Vallerie McLaughlin et al.   EUROPEAN RESPIRATORY JOURNAL

Fresolimumab treatment decreases biomarkers and improves clinical symptoms in systemic sclerosis patients

(2015) Lisa M. Rice et al.   JOURNAL OF CLINICAL INVESTIGATION

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

Reliability, validity and responsiveness to change of the Saint George’s Respiratory Questionnaire in early diffuse cutaneous systemic sclerosis

(2015) Beth Wallace et al.   RHEUMATOLOGY

Survival in systemic sclerosis–pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry

(2015) Monique Hinchcliff et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis

(2015) Yanjie Hao et al.   ARTHRITIS RESEARCH & THERAPY

Predictive Value of Serial High-Resolution Computed Tomography Analyses and Concurrent Lung Function Tests in Systemic Sclerosis

(2015) Anna-Maria Hoffmann-Vold et al.   Arthritis & Rheumatology

Endothelial Cells Expressing Endothelial and Mesenchymal Cell Gene Products in Lung Tissue From Patients With Systemic Sclerosis-Associated Interstitial Lung Disease

(2015) Fabian A. Mendoza et al.   Arthritis & Rheumatology

Brief Report: Pulmonary Function Tests: High Rate of False-Negative Results in the Early Detection and Screening of Scleroderma-Related Interstitial Lung Disease

(2015) Yossra A. Suliman et al.   Arthritis & Rheumatology

Survival of Adults With Systemic Sclerosis Following Lung Transplantation: A Nationwide Cohort Study

(2015) Elana J. Bernstein et al.   Arthritis & Rheumatology

Immunochip Analysis Identifies Multiple Susceptibility Loci for Systemic Sclerosis

(2014) Maureen D. Mayes et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Relationship between quantitative radiographic assessments of interstitial lung disease and physiological and clinical features of systemic sclerosis

(2014) Donald P Tashkin et al.   ANNALS OF THE RHEUMATIC DISEASES

Unique Predictors of Mortality in Patients With Pulmonary Arterial Hypertension Associated With Systemic Sclerosis in the REVEAL Registry

(2014) Lorinda Chung et al.   CHEST

Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study

(2014) O. Sitbon et al.   EUROPEAN RESPIRATORY JOURNAL

Autologous Hematopoietic Stem Cell Transplantation vs Intravenous Pulse Cyclophosphamide in Diffuse Cutaneous Systemic Sclerosis

(2014) Jacob M. van Laar et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Interstitial lung disease in connective tissue disease—mechanisms and management

(2014) Athol U. Wells et al.   Nature Reviews Rheumatology

Lung and heart-lung transplantation for systemic sclerosis patients. A monocentric experience of 13 patients, review of the literature and position paper of a multidisciplinary Working Group

(2014) David Launay et al.   PRESSE MEDICALE

Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study

(2014) Vivien M. Hsu et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

Borderline pulmonary arterial pressure in systemic sclerosis patients: a post-hoc analysis of the DETECT study

(2014) Scott H Visovatti et al.   ARTHRITIS RESEARCH & THERAPY

Derivation and Validation of a Prediction Rule for Two-Year Mortality in Early Diffuse Cutaneous Systemic Sclerosis

(2014) Robyn T. Domsic et al.   Arthritis & Rheumatology

Prediction of Pulmonary Complications and Long-Term Survival in Systemic Sclerosis

(2014) Svetlana I. Nihtyanova et al.   Arthritis & Rheumatology

Assessment of Brd4 Inhibition in Idiopathic Pulmonary Fibrosis Lung Fibroblasts and in Vivo Models of Lung Fibrosis

(2013) Xiaoyan Tang et al.   AMERICAN JOURNAL OF PATHOLOGY

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

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

Recommendations for Screening and Detection of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

(2013) Dinesh Khanna et al.   ARTHRITIS AND RHEUMATISM

Skin Gene Expression Correlates of Severity of Interstitial Lung Disease in Systemic Sclerosis

(2013) Shervin Assassi et al.   ARTHRITIS AND RHEUMATISM

Anticoagulation and Survival in Pulmonary Arterial Hypertension

(2013) Karen M. Olsson et al.   CIRCULATION

Serum Interleukin 6 Is Predictive of Early Functional Decline and Mortality in Interstitial Lung Disease Associated with Systemic Sclerosis

(2013) A. De Lauretis et al.   JOURNAL OF RHEUMATOLOGY

Targeting of αv integrin identifies a core molecular pathway that regulates fibrosis in several organs

(2013) Neil C Henderson et al.   NATURE MEDICINE

Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension

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

Proteome-wide Analysis and CXCL4 as a Biomarker in Systemic Sclerosis

(2013) Lenny van Bon et al.   NEW ENGLAND JOURNAL OF MEDICINE

Survival after lung transplantation in systemic sclerosis. A systematic review

(2013) Irfan Y. Khan et al.   RESPIRATORY MEDICINE

Microarray profiling reveals suppressed interferon stimulated gene program in fibroblasts from scleroderma-associated interstitial lung disease

(2013) Gisela E Lindahl et al.   RESPIRATORY RESEARCH

Cardiac arrhythmias and conduction defects in systemic sclerosis

(2013) Alessandra Vacca et al.   RHEUMATOLOGY

Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis

(2013) Carmel J Stock et al.   THORAX

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

Scleroderma pathogenesis: a pivotal role for fibroblasts as effector cells

(2013) Adrian J Gilbane et al.   ARTHRITIS RESEARCH & THERAPY

Editorial: Molecular Insights Into Systemic Sclerosis-Associated Interstitial Lung Disease

(2013) Richard M. Silver et al.   Arthritis & Rheumatology

BMP type II receptor deficiency confers resistance to growth inhibition by TGF-β in pulmonary artery smooth muscle cells: role of proinflammatory cytokines

(2012) Rachel J. Davies et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry

(2012) Sangmee Bae et al.   ANNALS OF THE RHEUMATIC DISEASES

Borderline Mean Pulmonary Artery Pressure in Patients With Systemic Sclerosis: Transpulmonary Gradient Predicts Risk of Developing Pulmonary Hypertension

(2012) Christopher J. Valerio et al.   ARTHRITIS AND RHEUMATISM

Exercise during cardiac catheterization distinguishes between pulmonary and left ventricular causes of dyspnea in systemic sclerosis patients

(2012) W. David Hager et al.   Clinical Respiratory Journal

Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy

(2012) Gregory J. Keir et al.   EUROPEAN RESPIRATORY JOURNAL

Association between the CTGF −945C/G polymorphism and systemic sclerosis: A meta-analysis

(2012) Xiufeng Zhang et al.   GENE

Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease

(2012) O. A. Moore et al.   RHEUMATOLOGY

A multicenter study confirms CD226 gene association with systemic sclerosis-related pulmonary fibrosis

(2012) Lara Bossini-Castillo et al.   ARTHRITIS RESEARCH & THERAPY

Improving the detection of pulmonary hypertension in systemic sclerosis using pulmonary function tests

(2011) Benjamin E. Schreiber et al.   ARTHRITIS AND RHEUMATISM

Clinical and Serological Hallmarks of Systemic Sclerosis Overlap Syndromes

(2011) A. PAKOZDI et al.   JOURNAL OF RHEUMATOLOGY

Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial

(2011) Richard K Burt et al.   LANCET

An Essential Role for Resident Fibroblasts in Experimental Lung Fibrosis Is Defined by Lineage-Specific Deletion of High-Affinity Type II Transforming Growth Factor β Receptor

(2010) Rachel K. Hoyles et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Reply

(2010) James R. Seibold et al.   ARTHRITIS AND RHEUMATISM

Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension

(2010) Eileen Hsu et al.   ARTHRITIS AND RHEUMATISM

Increased epithelial permeability in pulmonary fibrosis in relation to disease progression

(2010) N. S. L. Goh et al.   EUROPEAN RESPIRATORY JOURNAL

Systemic sclerosis and bilateral lung transplantation: a single centre experience

(2010) R. Saggar et al.   EUROPEAN RESPIRATORY JOURNAL

Cardiopulmonary Exercise Testing with Right-heart Catheterization in Patients with Systemic Sclerosis

(2010) A. J. WALKEY et al.   JOURNAL OF RHEUMATOLOGY

Clinical experience with bosentan and sitaxentan in connective tissue disease-associated pulmonary arterial hypertension

(2010) C. J. Valerio et al.   RHEUMATOLOGY

Gastroesophageal Reflux Incites Interstitial Lung Disease in Systemic Sclerosis: Clinical, Radiologic, Histopathologic, and Treatment Evidence

(2010) Romy B. Christmann et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

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

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

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

Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study

(2009) S.I. Nihtyanova et al.   QJM-AN INTERNATIONAL JOURNAL OF MEDICINE

A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis

(2009) S. I. Nihtyanova et al.   RHEUMATOLOGY

Interstitial Lung Disease in Systemic Sclerosis

(2008) Nicole S. L. Goh et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

A large multicentre analysis of CTGF -945 promoter polymorphism does not confirm association with systemic sclerosis susceptibility or phenotype

(2008) B Rueda et al.   ANNALS OF THE RHEUMATIC DISEASES

Association study of a polymorphism of the CTGF gene and susceptibility to systemic sclerosis in the Japanese population

(2008) Y Kawaguchi et al.   ANNALS OF THE RHEUMATIC DISEASES

Fibroblast-specific perturbation of transforming growth factor β signaling provides insight into potential pathogenic mechanisms of scleroderma-associated lung fibrosis: Exaggerated response to alveolar epithelial injury in a novel mouse model

(2008) Rachel K. Hoyles et al.   ARTHRITIS AND RHEUMATISM