The prognosis and management of reclassified systemic lupus erythematosus associated pulmonary arterial hypertension according to 2022 ESC/ERS guidelines.

Background And Aims: The 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guideline has recently revised the hemodynamic definition of pulmonary arterial hypertension. However, there is currently limited research on the prognosis and treatment of system lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH) patients that have been reclassified by the new hemodynamic definition. This study aims to analyze the prognosis of newly reclassified SLE-PAH patients and provide recommendations for the management strategy.

A prognostic model for systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study.

Background: Pulmonary 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.

Methods: A prognostic model was developed from a multicenter, longitudinal national cohort of consecutively evaluated patients with systemic lupus erythematosus-associated pulmonary arterial hypertension.

Association Study Identified HLA-DQA1 as a Novel Genetic Risk of Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension.

Objective: Pulmonary arterial hypertension (PAH) is a severe complication of systemic lupus erythematosus (SLE). However, the genetic signatures of SLE-associated PAH have not been well studied. We aimed to identify genetic variants implicated in SLE-associated PAH susceptibility within the major histocompatibility complex (MHC) region and assess the contribution to clinical outcomes.

The diagnostic and prognostic value of growth differentiation factor-15 in systemic lupus erythematosus-associated pulmonary arterial hypertension.

Growth-differentiation factor (GDF)-15 is a member of transforming growth factor-β-related cytokine and may respond to right ventricular overload. The objective of this article was to assess the diagnosis and prognostic value of GDF-15 in systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). Serum samples were obtained from 65 patients with SLE-PAH, 51 sex and age matched patients of SLE without PAH (SLE-non-PAH), and 32 healthy controls.

Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension.

Objective: Connective tissue disease associated pulmonary hypertension (CTD-PH) is classified as a subgroup of WHO group 1 PH, also called pulmonary arterial hypertension (PAH). However, not all CTD-PH fit hemodynamic definition of PAH. This study investigates the diversity of hemodynamic types of CTD-PH, their differences in clinical characteristics and outcomes.

Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study.

Objective: This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH).

Methods: A multicenter prospective cohort of SLE-associated PAH (CSTAR-PAH cohort) diagnosed based on right heart catheterization (RHC) was established. Baseline and follow-up records were collected.

Characteristics and risk factors of severe coronary artery disease in systemic lupus erythematosus: A multicenter, Chinese Rheumatism Date Center database study.

Aim: Systemic lupus erythematosus (SLE) with severe coronary artery disease (CAD) is associated with increased mortality. This study aimed to assess the characteristics and risk factors of severe CAD in SLE.

Method: This multicenter, cross-sectional study enrolled consecutive patients with SLE included in the Chinese Rheumatism Date Center registry.

Immunosuppressive therapy in patients with connective tissue disease-associated pulmonary arterial hypertension: A systematic review.

Objectives: It is currently accepted that inflammation plays an important role in the pathogenesis of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). However, the efficacy of immunosuppressive therapy remains anecdotal. The objective of this systematic review was to evaluate the efficacy of immunosuppressive therapy in patients with CTD-PAH and to further assess whether response differs between CTD subtypes and clinical features.

The treatment strategy of connective tissue disease associated pulmonary arterial hypertension: Evolving into the future.

Pulmonary arterial hypertension (PAH) is a frequent but severe vascular complication of patients with connective tissue diseases (CTDs) and a major cause of significant morbidity and mortality in these patients. Over the past few decades, effective therapies that targeting key signaling pathways involved in PAH have significantly improved patients symptoms and quality of life, and CTD-PAH patients are also greatly benefit from them. However, the current treatments fail to be completely curative, and prognosis of PAH patients remains poor.

Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: case series and literature review.

Objective: This study aimed to investigate the clinical characteristics and outcomes of pregnancy complicated by SLE-associated pulmonary arterial hypertension (SLE-PAH) in a case series and literature review.

Methods: This single-centre retrospective study included 10 consecutive pregnancies complicated by SLE-PAH confirmed by right heart catheterisation (RHC) at Peking Union Medical College Hospital between 2009 and 2020. A literature search was conducted and 14 pregnancy cases complicated by SLE-PAH were reviewed.

The role of anti-ribosomal P autoantibodies in the prediction of neuropsychiatric damage in systemic lupus erythematosus based on CSTAR cohort (XIV).

Objectives: To identify the predictive value of anti-ribosomal P protein (anti-RibP) antibodies on the accrual of neuropsychiatric damage in systemic lupus erythematosus (SLE) patients in a large cohort in the Chinese SLE Treatment and Research group (CSTAR) database.

Methods: This single-center prospective study was conducted based on data from the CSTAR registry. At baseline, we collected demographic characteristics, autoantibody profiles, clinical manifestations, disease activity status, and organ damage.

The LPS induced pyroptosis exacerbates BMPR2 signaling deficiency to potentiate SLE-PAH.

Pulmonary arterial hypertension (PAH) is a common and fatal complication of systemic lupus erythematosus (SLE). Whether the BMP receptor deficiency found in the genetic form of PAH is also involved in SLE-PAH patients remains to be identified. In this study, we employed patient-derived samples from SLE-associated PAH (SLE-PAH) and established comparable mouse models to clarify the role of BMP signaling in the pathobiology of SLE-PAH.

Ten-year survival analysis of patients with primary Sjögren's syndrome in China: a national prospective cohort study.

Aims: To investigate the long-term survival of patients with primary Sjögren's syndrome (pSS) in China.

Methods: Patients with pSS who fulfilled the 2002 American-European Consensus Group classification criteria were prospectively enrolled from 2004 to 2011. Their baseline clinical, laboratory, and therapeutic data were collected.

Validation of the REVEAL Prognostic Models in Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension.

No previous studies have investigated the predictive performance of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) prognostic equation and simplified risk score calculator in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). We aimed to validate these prediction tools in an external cohort of patients with SLE-PAH. In this study, the validation cohort consisted of patients with SLE-PAH registered in a prospective, multicenter, nationwide database between November 2006 and May2016.

Chinese SLE Treatment and Research Group Registry (CSTAR) XIII: prevalence and risk factors for chronic scarring alopecia in patients with systemic lupus erythematosus.

Background: Scarring alopecia in systemic lupus erythematosus (SLE) patients caused reduced life quality and prolonged disease course. This case-control study aims to survey the prevalence of scarring alopecia during the disease course of SLE and evaluate the risk factors for scarring alopecia in Chinese SLE patients.

Methods: SLE patients in Chinese SLE treatment and Research group (CSTAR) were recruited.

Clinical efficacy and safety of sirolimus in systemic lupus erythematosus: a real-world study and meta-analysis.

Objective: To provide real-world data and summarize current clinical evidence on the efficacy and safety of sirolimus in active systemic lupus erythematosus (SLE) patients.

Methods: This was a prospective real-world clinical study. Included SLE patients should have Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) ⩾ 2.

Pulmonary arterial hypertension associated with primary Sjögren's syndrome: a multicentre cohort study from China.

Objectives: Primary Sjögren's syndrome (pSS) is an important cause of pulmonary arterial hypertension (PAH), which remains insufficiently studied and needs attention. This study aimed to investigate the clinical characteristics, risk factors, prognosis and risk assessment of pSS-PAH.

Methods: We established a multicentre cohort of pSS-PAH diagnosed by right heart catheterisation.

IoT-RECSM-Resource-Constrained Smart Service Migration Framework for IoT Edge Computing Environment.

The edge-based computing paradigm (ECP) becomes one of the most innovative modes of processing distributed Interneit of Things (IoT) sensor data. However, the edge nodes in ECP are usually resource-constrained. When more services are executed on an edge node, the resources required by these services may exceed the edge node's, so as to fail to maintain the normal running of the edge node.

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: Baseline characteristics and risk factors.

Aim: Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)-confirmed SLE-associated PAH and identify risk factors for PAH in SLE patients.

Methods: A multicenter, cross-sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry.