ReviewOpen access rheumatology : research and reviews2017
Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies.
Review in Open access rheumatology : research and reviews, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 69 citations in OpenAlex.
- Lupus-Associated Pulmonary Arterial Hypertension: A Success Story.JACC. Case reports · 2025Article
- Insights into Pulmonary Arterial Hypertension in Connective Tissue Diseases.Journal of clinical medicine · 2025Review
- Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort.Lupus science & medicine · 2025Article
- Connective tissue disease-related interstitial lung disease and pulmonary hypertension.Breathe (Sheffield, England) · 2025Review
- Risk Factors for Adverse Outcomes in Connective Tissue Disease-Associated Pulmonary Hypertension.Reviews in cardiovascular medicine · 2025Review
- Exploring the Causal Link Between Autoimmune Diseases and Pulmonary Arterial Hypertension: A Bidirectional Mendelian Randomization Study.Global heart · 2025Article
- Diagnostic enigma of pulmonary hypertension: Tale from a series of three patients.Medical journal, Armed Forces India · 2024Article
- A case report of systemic lupus erythematosus with severe pulmonary hypertension presenting as large pericardial effusion with early signs of cardiac tamponade: a diagnostic and therapeutic challenge.European heart journal. Case reports · 2024Article
- The role of immune cells in the pathogenesis of connective tissue diseases-associated pulmonary arterial hypertension.Frontiers in immunology · 2024Review
- Epidemiology of systemic lupus erythematosus in Korea.Journal of rheumatic diseases · 2023Review
- Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment.Clinical rheumatology · 2023Review
- Clinical characteristics and prognosis in systemic lupus erythematosus-associated pulmonary arterial hypertension based on consensus clustering and risk prediction model.Arthritis research & therapy · 2023Article
- Systemic Lupus Erythematosus and Pulmonary Hypertension.International journal of molecular sciences · 2023Review
- 2023 Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) Joint Consensus on Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.Acta Cardiologica Sinica · 2023Article
- Thoracic Involvement in Systemic Autoimmune Rheumatic Diseases: Pathogenesis and Management.Clinical reviews in allergy & immunology · 2022Review
- Anesthetic management of cesarean delivery of parturient with systemic lupus erythematosus associated with pulmonary arterial hypertension - A case report.Anesthesia and pain medicine · 2022Article
- Article
- Pediatric Lupus Presenting as Pulmonary Hypertension, Myocarditis, and Massive Pericardial Effusion in an 11-Year-Old Girl: A Case Report and Literature Review.Frontiers in pediatrics · 2022Article
- Respiratory Manifestations in Systemic Lupus Erythematosus.Pharmaceuticals (Basel, Switzerland) · 2021Review
- Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?Breathe (Sheffield, England) · 2020Review
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud's phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.