Clinical features of pulmonary arterial hypertension associated with systemic sclerosis

Systemic sclerosis is an autoimmune disorder of the connective tissue characterized by disordered inflammation and fibrosis leading to skin thickening and visceral organ complications. Pulmonary involvement, in the form of pulmonary arterial hypertension and/or interstitial lung disease, is the leading cause of morbidity and mortality among individuals with scleroderma. There are no disease-specific therapies for pulmonary involvement of scleroderma, and pulmonary arterial hypertension in this cohort has typically been associated with worse outcomes and less clinical response to modern therapy compared to other forms of Group I pulmonary hypertension in the classification from the World Symposium on Pulmonary Hypertension. Ongoing research aims to delineate how pathologic microvascular remodeling and fibrosis contribute to this poor response and offer a window into future therapeutic targets.

• Publication year

  2023

• Venue

  Frontiers in Medicine

• Publication date

  2023-09-27

• Fields of study

  Medicine

• Identifiers
  DOI 10.3389/fmed.2023.1264906PMID 37828949PMCID 10565655
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• State-of-the-art evidence in the treatment of systemic sclerosis

  2023cited by this paper
• Minimal Clinically Important Difference in the Six-Minute Walk Distance for Patients with Pulmonary Arterial Hypertension.

  2023cited by this paper
• Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension.

  2023cited by this paper
• 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

  2022cited by this paper
• Increased risk of mortality in systemic sclerosis-associated pulmonary hypertension: a systemic review and meta-analysis

  2022cited by this paper
• Improved Survival for Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension.

  2022cited by this paper
• Safety and efficacy of rituximab in systemic sclerosis (DESIRES): open-label extension of a double-blind, investigators-initiated, randomised, placebo-controlled trial.

  2022cited by this paper
• A Systemic Review and Meta-analysis of the Effect of SARS-CoV-2 Infection on Sperm Parameters

  2022cited by this paper
• Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation.

  2021cited by this paper
• Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders

  2021cited by this paper
• Polysomnography findings and risk factors for sleep-disordered breathing in patients with systemic sclerosis

  2021cited by this paper
• Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease.

  2021influential reference
• Scleroderma epidemiology update

  2021cited by this paper
• Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis

  2021cited by this paper
• 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.

  2021cited by this paper
• Outcomes of pulmonary vasodilator use in Veterans with pulmonary hypertension associated with left heart disease and lung disease

  2021cited by this paper
• Pulmonary Arterial Hypertension.

  2021cited by this paper
• Scleroderma and obstructive sleep apnea: a consideration of immunological aspects and the role of fibrosis

  2021cited by this paper
• Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc).

  2020cited by this paper
• Increased frequency of obstructive sleep apnea in the patients with systemic sclerosis

  2020cited by this paper
• Impact of concomitant obstructive sleep apnea on pulmonary involvement and main pulmonary artery diameter in adults with scleroderma

  2020cited by this paper
• Gender-related differences in systemic sclerosis.

  2020cited by this paper
• Scleroderma

  2020cited by this paper
• Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease

  2020cited by this paper
• Haemodynamic definitions and updated clinical classification of pulmonary hypertension

  2019cited by this paper
• Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature

  2019cited by this paper
• GWAS for systemic sclerosis identifies multiple risk loci and highlights fibrotic and vasculopathy pathways

  2019cited by this paper
• Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension

  2019cited by this paper
• Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

  2019cited by this paper
• Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis–Associated Versus Idiopathic Pulmonary Arterial Hypertension

  2018influential reference
• Pulmonary Veno-Occlusive Disease

  2018cited by this paper
• Prevalence of auto-antibodies associated to pulmonary arterial hypertension in scleroderma - A review.

  2018cited by this paper
• Insights into myofibroblasts and their activation in scleroderma: opportunities for therapy?

  2018cited by this paper
• A comprehensive systematic review and meta-analysis of pharmacological and dietary supplement interventions in paediatric autism: moderators of treatment response and recommendations for future research

  2017cited by this paper
• Lung Transplant Outcomes in Systemic Sclerosis with Significant Esophageal Dysfunction. A Comprehensive Single-Center Experience.

  2016cited by this paper
• Pulmonary veno-occlusive disease

  2016cited by this paper
• Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2

  2016cited by this paper
• Aspiration-related pulmonary syndromes.

  2015influential reference
• Genetics, Epigenetics, and Genomics of Systemic Sclerosis.

  2015cited by this paper
• Ambrisentan and Tadalafil Up-front Combination Therapy in Scleroderma-associated Pulmonary Arterial Hypertension.

  2015cited by this paper
• The Right Ventricle in Scleroderma (2013 Grover Conference Series)

  2015cited by this paper
• Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

  2015cited by this paper
• Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial.

  2014cited by this paper
• Improved Transplant‐Free Survival in Patients With Systemic Sclerosis–Associated Pulmonary Hypertension and Interstitial Lung Disease

  2014cited by this paper
• Right Ventricular Dysfunction in Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

  2013cited by this paper
• Systemic sclerosis-associated pulmonary arterial hypertension.

  2013cited by this paper
• 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative

  2013cited by this paper
• Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study

  2013cited by this paper
• 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.

  2013cited by this paper
• Survival after lung transplantation in systemic sclerosis. A systematic review.

  2013cited by this paper
• Riociguat for the treatment of pulmonary arterial hypertension.

  2013cited by this paper
• An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative

  2013cited by this paper
• Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension.

  2012cited by this paper
• Lung involvement in systemic sclerosis.

  2011influential reference
• Pulmonary arterial hypertension associated with systemic sclerosis

  2011cited by this paper
• Right and left ventricular myocardial perfusion reserves correlate with right ventricular function and pulmonary hemodynamics in patients with pulmonary arterial hypertension.

  2011cited by this paper
• Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study

  2010cited by this paper
• Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension.

  2010cited by this paper
• Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype.

  2010cited by this paper
• Heritability of vasculopathy, autoimmune disease, and fibrosis in systemic sclerosis: a population-based study.

  2010cited by this paper
• Systemic sclerosis-associated pulmonary arterial hypertension.

  2010cited by this paper
• Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era.

  2009cited by this paper
• Scleroderma.

  2009cited by this paper
• Pulmonary veno-occlusive disease

  2009cited by this paper
• Guidelines for the diagnosis and treatment of pulmonary hypertension

  2009cited by this paper
• Comparison of baseline characteristics and survival between patients with idiopathic and connective tissue disease-related pulmonary arterial hypertension.

  2009cited by this paper
• Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease.

  2009cited by this paper
• Tadalafil Therapy for Pulmonary Arterial Hypertension

  2009cited by this paper
• Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy

  2009cited by this paper
• Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension

  2009influential reference
• The heart in scleroderma.

  2008cited by this paper
• The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement

  2008cited by this paper
• Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension.

  2008cited by this paper
• Changes in causes of death in systemic sclerosis, 1972–2002

  2007cited by this paper
• Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases.

  2007cited by this paper
• Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

  2007cited by this paper
• Clinical differences between idiopathic and scleroderma-related pulmonary hypertension.

  2006cited by this paper
• Pulmonary arterial hypertension in France: results from a national registry.

  2006cited by this paper
• Isolated pulmonary hypertension in scleroderma

  2005cited by this paper
• Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension

  2005cited by this paper
• Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.

  2005cited by this paper
• Sildenafil citrate therapy for pulmonary arterial hypertension.

  2005cited by this paper
• Pulmonary Veno-Occlusive Disease

  2005cited by this paper
• Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease.

  2004cited by this paper
• Following the Molecular Pathways toward an Understanding of the Pathogenesis of Systemic Sclerosis

  2004cited by this paper
• Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study.

  2003cited by this paper
• Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial.

  2002cited by this paper
• Primary Pulmonary Hypertension Is Associated With Reduced Pulmonary Vascular Expression of Type II Bone Morphogenetic Protein Receptor

  2002cited by this paper
• Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts.

  2001cited by this paper
• Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene.

  2000cited by this paper
• Continuous Intravenous Epoprostenol for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease

  2000cited by this paper
• Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial.

  2000cited by this paper
• Heterozygous germline mutations in BMPR2, encoding a TGF-β receptor, cause familial primary pulmonary hypertension

  2000cited by this paper
• [Phase II study].

  1995cited by this paper
• Scleroderma (systemic sclerosis): classification, subsets and pathogenesis.

  1988cited by this paper
• The randomized clinical trial.

  1972cited by this paper

• Bridging knowledge gaps: A case report of pulmonary Veno-occlusive disease in systemic sclerosis

  2025cites this paper
• Thiazolidine derivative (LPSF/CR-35) modulates the production of IL-17A, IL-10, IL-4, TFN, CCL2 and CXCL8 in PBMC from systemic sclerosis patients

  2025cites this paper
• Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: From Bedside to Bench and Back Again

  2024cites this paper
• IFIT3 mediates TBK1 phosphorylation to promote activation of pDCs and exacerbate systemic sclerosis in mice

  2024cites this paper
• Hipertensão pulmonar na esclerose sistêmica: investigação e diagnóstico

  2024cites this paper