ArticleRespiratory research2023
A prognostic model for systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study.
Article in Respiratory research, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07653958 (Research on the Clinical Risk Factor Assessment System for Acute Pulmonary Hypertension Crisis and Its Mortality), which is not on this map. Cited by 7 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Research on the Clinical Risk Factor Assessment System for Acute Pulmonary Hypertension Crisis and Its Mortality
Who cites it
7 citing papers in PubMed, 7 citations in OpenAlex.
- The clinical characteristics and risk factors of pulmonary arterial hypertension in primary Sjögren disease: a single-center retrospective study.Clinical rheumatology · 2026Article
- Clinical, serological and haemodynamic factors associated with poor outcomes in systemic lupus erythematosus-associated pulmonary arterial hypertension: a multicentre retrospective study from Korea.Rheumatology (Oxford, England) · 2026Article
- Article
- RareInnovation (Cambridge (Mass.)) · 2026Article
- Implications of systolic pulmonary artery pressure trajectories in systemic lupus erythematosus-associated pulmonary hypertension: a CSTAR-PAH cohort study.Frontiers in immunology · 2026Article
- Pulmonary Involvement in Systemic Lupus Erythematosus: A Potentially Overlooked Condition.Biomedicines · 2025Review
- First nomogram for predicting interstitial lung disease and pulmonary arterial hypertension in SLE: a machine learning approach.Respiratory research · 2025Article
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Authors and funding
24 authors at 15 institutions in 1 country.
Funding
Abstract
backgroundPulmonary arterial hypertension is a major cause of death in systemic lupus erythematosus, but there are no tools specialized for predicting survival in systemic lupus erythematosus-associated pulmonary arterial hypertension. RESEARCH QUESTION: To develop a practical model for predicting long-term prognosis in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension.
methodsA prognostic model was developed from a multicenter, longitudinal national cohort of consecutively evaluated patients with systemic lupus erythematosus-associated pulmonary arterial hypertension. The study was conducted between November 2006 and February 2020. All-cause death was defined as the endpoint. Cox regression and least absolute shrinkage and selection operators were used to fit the model. Internal validation of the model was assessed by discrimination and calibration using bootstrapping.
resultsOf 310 patients included in the study, 81 (26.1%) died within a median follow-up of 5.94 years (interquartile range 4.67-7.46). The final prognostic model included eight variables: modified World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, estimated glomerular filtration rate, thrombocytopenia, mild interstitial lung disease, N-terminal pro-brain natriuretic peptide/brain natriuretic peptide level, and direct bilirubin level. A 5-year death probability predictive algorithm was established and validated using the C-index (0.77) and a satisfactory calibration curve. Risk stratification was performed based on the predicted probability to improve clinical decision-making.
conclusionsThis new risk stratification model for systemic lupus erythematosus-associated pulmonary arterial hypertension may provide individualized prognostic probability using readily obtained clinical risk factors. External validation is required to demonstrate the accuracy of this model's predictions in diverse patient populations.
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