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  • Wiley  (15)
  • Wang, Qian  (15)
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  • 1
    Online Resource
    Online Resource
    Mortality and prognostic factors in connective tissue disease‐associated pulmonary arterial hypertension patients complicated with right heart failure
    Wiley ; 2023
    In:  International Journal of Rheumatic Diseases Vol. 26, No. 5 ( 2023-05), p. 862-869
    Details
    In: International Journal of Rheumatic Diseases, Wiley, Vol. 26, No. 5 ( 2023-05), p. 862-869
    Abstract: To identify predictive factors associated with mortality in connective tissue disease‐associated pulmonary arterial hypertension (CTD‐PAH) patients who were complicated with right heart failure (RHF). Methods In this single‐center retrospective study, baseline demographics, clinical features, laboratory results, and hemodynamic assessments were collected. Kaplan–Meier analysis was applied to analyze all‐cause mortality. Univariate and forward stepwise multivariate Cox proportional regression analyses were performed to identify independent predictors of mortality. Results A total of 51 right heart catheterization‐confirmed CTD‐PAH patients complicated with RHF were consecutively enrolled in this study from 2012 to 2022. Forty‐eight (94%) enrolled patients were female and the mean age was 36.0 ± 11.8 years. Thirty‐two (61.5%) were systemic lupus erythematosus‐PAH and 33%/67% showed World Health Organization functional class III/IV, respectively. Twenty‐five (49%) of those patients died and Kaplan–Meier analysis showed the overall 1‐, 3‐, and 5‐week survival rates from the time of hospitalization as 86.28%, 60.78%, and 56.86%, respectively. RHF in CTD‐PAH patients mainly resulted from progression of PAH ( n  = 19) and infection ( n  = 5), which also contributed to the leading causes of death. Statistical analysis between survivors and non‐survivors showed that death from RHF was associated with higher levels of urea (9.66 vs 6.34 mmol/L, P  = 0.002), lactate (cLac: 2.65 vs 1.9 mmol/L, P  = 0.006), total bilirubin (23.1 vs 16.9 μmol/L, P  = 0.018) and direct bilirubin (10.5 vs 6.5 μmol/L, P  = 0.004), but with lower levels of hematocrit (33.7 vs 39, P  = 0.004), cNa+ (131 vs 136 mmol/L, P  = 0.003). Univariate and forward stepwise multivariate Cox proportional regression analyses indicated that the level of cLac (hazards ratio:1.297; 95% CI: 1.076–1.564; P  = 0.006) was an independent risk factor for mortality. Conclusion The short‐term prognosis of CTD‐PAH complicated with RHF was very poor, and hyperlactic acidemia (cLac  〉  2.85 mmoL/L) was an independent predicting factor for mortality of CTD‐PAH patients complicated with RHF.
    Type of Medium: Online Resource
    ISSN: 1756-1841 , 1756-185X
    URL: Issue
    URL: Article
    DOI: 10.1111/apl.v26.5
    DOI: 10.1111/1756-185X.14660
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 2427877-4
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  • 2
    Online Resource
    Online Resource
    Chinese registry of rheumatoid arthritis (CREDIT): III. The transition of disease activity during follow‐ups and predictors of achieving treatment target
    Wiley ; 2020
    In:  International Journal of Rheumatic Diseases Vol. 23, No. 12 ( 2020-12), p. 1719-1727
    Details
    In: International Journal of Rheumatic Diseases, Wiley, Vol. 23, No. 12 ( 2020-12), p. 1719-1727
    Abstract: The Chinese Registry of Rheumatoid Arthritis (CREDIT) is the first nationwide multi‐center prospective rheumatoid arthritis (RA) registration cohort in China. This study aimed at presenting disease activities transition during follow‐ups and identifying predictors to treatment response. Method Patients who had baseline, 3‐ and 6‐month follow‐up data from November 2016 to April 2018 were recruited. Then, we selected patients who did not reach remission (REM)/low disease activity (LDA) at baseline to investigate the predictors for treatment response. Results There were 979 patients included (83.00% female, mean age 51.8 and median duration 3.84 years). REM/LDA rate at baseline, 3‐, and 6‐month follow‐up were 34.02%, 59.35% and 68.23%. Additionally, early RA has more chance to achieve targets than established RA (6 months: 59.79% vs 48.13%, P  = .002). High baseline Disease Activity Score of 28 joints (DAS28) (early RA: odds ratio [OR] 1.319, P  = .019; established RA: OR 1.337, P   〈  .001), biologic disease‐modifying anti‐rheumatic drugs (bDMARD)/targeted synthetic DMARD combined conventional DMARD therapy (early RA: OR 9.023, P  = .046) and prednisolone usage (early RA: OR 2.526, P   〈  .001) are positively associated with Clinical Disease Assessment Index (CDAI) decreasing at 3 months. Low baseline DAS28 (REM/LDA: early RA: OR 0.650, P  〈  .001; established RA: OR 0.612, P   〈  .001. REM: early RA: OR 0.743, P  = .021; established RA: OR 0.674, P   〈  .001), young age (REM: early RA: OR 0.977, P  = .048) and decrease of CDAI at 3 months (REM/LDA: early RA: OR 7.185, P   〈  .001; established RA: OR 8.752, P   〈  .001. REM: early RA: OR 5.602, P   〈  .001; established RA: OR 4.955, P   〈  .001) predict REM/LDA at 6 months. Conclusion Disease activity decreased during follow‐ups. Disease duration, baseline disease activity, age, treatment strategies, and CDAI decreasing were associated with treatment response.
    Type of Medium: Online Resource
    ISSN: 1756-1841 , 1756-185X
    URL: Issue
    URL: Article
    DOI: 10.1111/apl.v23.12
    DOI: 10.1111/1756-185X.13996
    Language: English
    Publisher: Wiley
    Publication Date: 2020
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  • 3
    Online Resource
    Online Resource
    Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors
    Wiley ; 2019
    In:  International Journal of Rheumatic Diseases Vol. 22, No. 5 ( 2019-05), p. 921-928
    Details
    In: International Journal of Rheumatic Diseases, Wiley, Vol. 22, No. 5 ( 2019-05), p. 921-928
    Abstract: 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. Baseline data for patients with SLE‐associated PAH and SLE patients without PAH were collected and compared. Risk factors for PAH among patients with SLE were identified. Results A total of 292 patients with SLE‐associated PAH were enrolled. RHC was used to reveal hemodynamic features, including mean pulmonary arterial pressure (46.2 ± 12.0 mm Hg), pulmonary arterial wedge pressure (7.84 ± 3.92 mm Hg), pulmonary vascular resistance (10.86 ± 5.57 Wood units), and cardiac index (2.77 ± 0.91 L/min × m 2 ). A multivariate logistic regression analysis showed that serositis (odds ratio [OR] = 5.524, 95% CI 3.605‐8.465, P   〈  0.001), anti‐ribonucleoprotein (RNP) antibody positivity (OR = 13.332, 95% CI 9.500‐18.710, P   〈  0.001), and diffusion capacity of carbon monoxide in the lung (DLCO)/%Pred 〈 70% (OR = 10.018, 95% CI 6.619‐15.162, P   〈  0.001) were independent predictors of PAH. We recommend using transthoracic echocardiography (TTE) to perform early screening of SLE patients who have serositis, anti‐RNP antibody positivity, or DLCO/%Pred 〈 70%. RHC is suggested for patients suspected of having PAH. Once a diagnosis of SLE‐PAH is confirmed, evaluation and treatment should immediately begin. Conclusion Overall, we recommend performing early screening using TTE in SLE patients with serositis, anti‐RNP antibodies, or a DLCO/%Pred 〈 70%, even for patients in a relatively stable condition according to SLE disease activity index.
    Type of Medium: Online Resource
    ISSN: 1756-1841 , 1756-185X
    URL: Issue
    URL: Article
    DOI: 10.1111/apl.2019.22.issue-5
    DOI: 10.1111/1756-185X.13478
    Language: English
    Publisher: Wiley
    Publication Date: 2019
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  • 4
    Online Resource
    Online Resource
    Predicting the Risk of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: A Chinese Systemic Lupus Erythematosus Treatment and Research Group Cohort Study
    Wiley ; 2021
    In:  Arthritis & Rheumatology Vol. 73, No. 10 ( 2021-10), p. 1847-1855
    Details
    In: Arthritis & Rheumatology, Wiley, Vol. 73, No. 10 ( 2021-10), p. 1847-1855
    Abstract: Pulmonary arterial hypertension (PAH) is a life‐threatening complication of systemic lupus erythematosus (SLE). However, there is no algorithm to identify those at high risk. This study was undertaken to develop a prediction model for PAH in patients with lupus that provides individualized risk estimates. Methods A multicenter, longitudinal cohort study was undertaken from January 2003 to January 2020. The study collected data on 3,624 consecutively evaluated patients diagnosed as having SLE. The diagnosis of PAH was confirmed by right‐sided heart catheterization. Cox proportional hazards regression and least absolute shrinkage and selection operator were used to fit the model. Model discrimination, calibration, and decision curve analysis were performed for validation. Results Ninety‐two lupus patients (2.54%) developed PAH during a median follow‐up of 4.84 years (interquartile range 2.42–8.84). The final prediction model included 5 clinical variables (acute/subacute cutaneous lupus, arthritis, renal disorder, thrombocytopenia, and interstitial lung disease) and 3 autoantibodies (anti‐RNP, anti‐Ro/SSA and anti‐La/SSB). A 10‐year PAH probability‐predictive nomogram was established. The model was internally validated by Harrell’s concordance index (0.78), the Brier score (0.03), and a satisfactory calibration curve. According to the net benefit and predicted probability thresholds, we recommend annual screening in high‐risk ( 〉 4.62%) lupus patients. Conclusion We developed a risk stratification model using routine clinical assessments. This new tool may effectively predict the future risk of PAH in patients with SLE.
    Type of Medium: Online Resource
    ISSN: 2326-5191 , 2326-5205
    URL: Issue
    URL: Article
    DOI: 10.1002/art.v73.10
    DOI: 10.1002/art.41740
    RVK:
    XA 10000
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2754614-7
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  • 5
    Online Resource
    Online Resource
    Association study identified HLA‐DQA1 as a novel genetic risk of systemic lupus erythematosus‐associated pulmonary arterial hypertension
    Wiley ; 2023
    In:  Arthritis & Rheumatology
    Details
    In: Arthritis & Rheumatology, Wiley
    Abstract: Pulmonary arterial hypertension (PAH) is 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. Methods 172 SLE‐associated PAH patients confirmed by right heart catheterization, 1303 SLE patients without PAH and 9906 healthy controls were included. Deep sequencing of the MHC region was performed to identify alleles, single‐nucleotide polymorphisms and amino acids. We compared SLE‐associated PAH patients with SLE patients without PAH and healthy controls. Clinical association study was conducted to explore the contribution to phenotypes. Results A total of 19881 genetic variants were identified within the MHC region. HLA‐DQA1 *03:02 was identified as a novel genetic variant associated with SLE‐associated PAH in the discovery cohort ( P =5.68×10 ‐12 ) and authenticated in an independent replication cohort ( P =1.30×10 ‐9 ). The strongest associated amino acid position was mapped to HLA‐DQα1 in the region affecting MHC/peptide‐CD4 + T‐cell receptor affinity and antigen binding. Clinical association study demonstrated that SLE‐associated PAH patients with HLA‐DQA1 *03:02 had significantly lower rates of target role achievement ( P =0.005) and survival ( P =0.04). Conclusion This study, based on the largest cohort of SLE‐associated PAH, is the first to investigate MHC region genetic variants contribute to SLE‐associated PAH susceptibility. HLA‐DQA1 *03:02 is a novel genetic risk factor and a prognostic factor in SLE‐associated PAH. SLE patients with this allele require regular monitoring and careful follow‐up for early diagnosis and interventions for potential PAH. This article is protected by copyright. All rights reserved.
    Type of Medium: Online Resource
    ISSN: 2326-5191 , 2326-5205
    URL: Article
    DOI: 10.1002/art.42641
    RVK:
    XA 10000
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2023
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  • 6
    Online Resource
    Online Resource
    Anti–Endothelin Receptor Type A Autoantibodies in Systemic Lupus Erythematosus–Associated Pulmonary Arterial Hypertension
    Wiley ; 2015
    In:  Arthritis & Rheumatology Vol. 67, No. 9 ( 2015-09), p. 2394-2402
    Details
    In: Arthritis & Rheumatology, Wiley, Vol. 67, No. 9 ( 2015-09), p. 2394-2402
    Abstract: To investigate the role of autoantibodies against endothelin 1 receptor type A (ETRA) in patients with systemic lupus erythematosus (SLE)–associated pulmonary arterial hypertension (PAH) and to examine the possibility that the pathogenesis of this disease is mediated by these autoantibodies. Methods ETRA autoantibodies in serum from patients with SLE‐associated PAH and serum from controls (SLE patients without PAH) were detected via a human ETRA epitope peptide–based enzyme‐linked immunosorbent assay. An exploratory cohort of patients with SLE‐associated PAH (n = 76) and an independent validation cohort of patients with SLE‐associated PAH confirmed by right‐sided heart catheterization (RHC) (n = 82) were enrolled. The clinical relevance of ETRA autoantibodies in SLE‐associated PAH was analyzed. The proliferation of vascular smooth muscle cells (SMCs) and the permeability of endothelial cells (ECs) were assessed in vitro in cells stimulated with polyclonal ETRA IgG autoantibodies. Expression of PAH‐related markers, i.e., serotonin transporter, platelet‐derived growth factor receptor β, vascular endothelial growth factor A, and platelet‐derived growth factor B was measured by quantitative polymerase chain reaction. In addition, a suboptimal dose of monocrotaline was used to induce PAH in rats, and the effect of ETRA autoantibodies in vivo was determined using a right ventricular hypertrophy index, pulmonary angiography, and laboratory parameters. Results ETRA autoantibodies occurred more frequently in SLE‐associated PAH (41.5%) than in controls (17.1%). There was a significant correlation between ETRA autoantibody titers and pulmonary artery systolic pressure measured by echocardiography (r = 0.2978, P  = 0.0038) or pulmonary artery systolic pressure measured by RHC (r = 0.2159, P  = 0.0257) in SLE‐associated PAH. ETRA autoantibodies could promote SMC proliferation, disrupt the endothelial barrier, and up‐regulate expression of PAH‐related markers, which could be blocked in the presence of an endothelin receptor antagonist. ETRA autoantibodies aggravated right ventricular hypertrophy and vascular remodeling in vivo. Conclusion We identified ETRA autoantibodies as a biomarker of mechanistic relevance in SLE. These autoantibodies may mediate PAH development in SLE.
    Type of Medium: Online Resource
    ISSN: 2326-5191 , 2326-5205
    URL: Issue
    URL: Article
    DOI: 10.1002/art.v67.9
    DOI: 10.1002/art.39212
    RVK:
    XA 10000
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2015
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  • 7
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    Online Resource
    Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome
    Wiley ; 2018
    In:  International Journal of Rheumatic Diseases Vol. 21, No. 5 ( 2018-05), p. 1068-1075
    Details
    In: International Journal of Rheumatic Diseases, Wiley, Vol. 21, No. 5 ( 2018-05), p. 1068-1075
    Abstract: To describe baseline characteristics of patients with primary Sjögren's syndrome ( pSS ) with right heart catheterization ( RHC )‐confirmed pulmonary arterial hypertension ( PAH ) and explore risk factors for PAH in pSS . Methods This case–control study included consecutive patients hospitalized with pSS – PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American‐European Consensus Group classification criteria for pSS – PAH was defined according to RHC ‐based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were analyzed by univariate binary logistic regression to identify possible risk factors for PAH. Results Twenty‐nine patients with RHC ‐confirmed pSS ‐ PAH were included (mean age at onset, 38.4 ± 8.3 years; mean pSS duration, 54.6 months). PAH was the initial manifestation of pSS in 12 patients (41.4%), and shortness of breath was the most common symptom (29/29, 100%). Mean pulmonary arterial pressure was 51.8 ± 10.0 mmHg, mean cardiac index was 2.3 ± 0.8 L/min/m 2 , and mean pulmonary vascular resistance was 13.0 ± 6.0 Wood units in this group. Treatments included immunosuppressive agents (93.1%) and PAH ‐targeted therapies (86.2%). We identified four independent risk factors for PAH in pSS : Raynaud's phenomenon (odds ratio [ OR ] = 9.660, P = 0.000), rheumatoid factor ≥ 200 U/ mL ( OR = 6.691, P = 0.001), hepatic injury ( OR = 3.284, P = 0.008) and pericardial effusion ( OR = 3.279, P = 0.016). Conclusions PAH can be the first manifestation of pSS . The pSS patients with Raynaud's phenomenon, high‐titer rheumatoid factor, hepatic injury or pericardial effusion should be screened for PAH.
    Type of Medium: Online Resource
    ISSN: 1756-1841 , 1756-185X
    URL: Issue
    URL: Article
    DOI: 10.1111/apl.2018.21.issue-5
    DOI: 10.1111/1756-185X.13290
    Language: English
    Publisher: Wiley
    Publication Date: 2018
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  • 8
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    Online Resource
    Identification of 6 dermatomyositis subgroups using principal component analysis‐based cluster analysis
    Wiley ; 2019
    In:  International Journal of Rheumatic Diseases Vol. 22, No. 8 ( 2019-08), p. 1383-1392
    Details
    In: International Journal of Rheumatic Diseases, Wiley, Vol. 22, No. 8 ( 2019-08), p. 1383-1392
    Abstract: Dermatomyositis (DM) is a heterogeneous disease with a wide range of clinical manifestations. The aim of the present study was to identify the clinical subtypes of DM by applying cluster analysis. Methods We retrospectively reviewed the medical records of 720 DM patients and selected 21 variables for analysis, including clinical characteristics, laboratory findings, and comorbidities. Principal component analysis (PCA) was first conducted to transform the 21 variables into independent principal components. Patient classification was then performed using cluster analysis based on the PCA‐transformed data. The relationships among the clinical variables were also assessed. Results We transformed the 21 clinical variables into nine independent principal components by PCA and identified six distinct subgroups. Cluster A was composed of two sub‐clusters of patients with classical DM and classical DM with minimal organ involvement. Cluster B patients were older and had malignancies. Cluster C was characterized by interstitial lung disease (ILD), skin ulcers, and minimal muscle involvement. Cluster D included patients with prominent lung, muscle, and skin involvement. Cluster E contained DM patients with other connective tissue diseases. Cluster F included all patients with myocarditis and prominent myositis and ILD. We found significant differences in treatment across the six clusters, with clusters E, C and D being more likely to receive aggressive immunosuppressive therapy. Conclusion We applied cluster analysis to a large group of DM patients and identified 6 clinical subgroups, underscoring the need for better phenotypic characterization to help develop individualized treatments and improve prognosis.
    Type of Medium: Online Resource
    ISSN: 1756-1841 , 1756-185X
    URL: Issue
    URL: Article
    DOI: 10.1111/apl.v22.8
    DOI: 10.1111/1756-185X.13609
    Language: English
    Publisher: Wiley
    Publication Date: 2019
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  • 9
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    Online Resource
    The diagnostic and prognostic value of growth differentiation factor‐15 in systemic lupus erythematosus‐associated pulmonary arterial hypertension
    Wiley ; 2023
    In:  Pulmonary Circulation Vol. 13, No. 1 ( 2023-01)
    Details
    In: Pulmonary Circulation, Wiley, Vol. 13, No. 1 ( 2023-01)
    Abstract: 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. Serum GDF‐15 level was detected by enzyme‐linked immunosorbent assay and the optimal cut‐off point was determined by receiver operating characteristic curve. The primary end‐point was death from any cause and the secondary end‐point was target goal achievement (TGA). Cox regression analyses and Kaplan−Meier method were performed to identify the prognostic value of GDF‐15. Serum GDF‐15 levels were significantly higher in SLE‐PAH patients (1112.14 ± 781.80 pg/mL) than SLE‐non‐PAH patients (810 ± 408 pg/mL) and healthy controls (442 ± 139 pg/mL) at baseline. The optimal cut‐off value of GDF‐15 in the diagnosis of SLE‐PAH was 733 pg/mL (AUC = 0.84). In patients with SLE‐PAH, GDF‐15 level was associated with 6 min walking distance ( ρ  = −0.385, p  = 0.017) and higher serum N terminal‐pro brain natriuretic peptide (NT‐proBNP) ( ρ  = 0.605, p   〈  0.001). Patients with GDF‐15  〉  733 pg/mL were more likely to death (adjusted hazard ratio [HR] = 4.01, 95% confidence intervals [CI] : 1.23−6.27, p  = 0.041) and less likely to achieve treatment goal (adjusted HR = 0.57, 95% CI: 0.23−0.79, p  = 0.028). In addition, patients with simultaneous elevation of GDF‐15 and NT‐proBNP showed lower proportion of TGA ( p  = 0.046). In conclusion, GDF‐15 is a new and promising biomarker of development and prognosis in SLE‐PAH. The combination of GDF‐15 and NT‐proBNP may provide more accurate prognostic information.
    Type of Medium: Online Resource
    ISSN: 2045-8940 , 2045-8940
    URL: Issue
    URL: Article
    DOI: 10.1002/pul2.v13.1
    DOI: 10.1002/pul2.12195
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 2638089-4
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  • 10
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    The Role of Anti–U1 RNP Positivity in Predicting Survival in Patients With Connective Tissue Disease–Associated Pulmonary Arterial Hypertension: Angel or Demon? Comment on the Article by Sobanski et al
    Wiley ; 2016
    In:  Arthritis & Rheumatology Vol. 68, No. 7 ( 2016-07), p. 1788-1789
    Details
    In: Arthritis & Rheumatology, Wiley, Vol. 68, No. 7 ( 2016-07), p. 1788-1789
    Type of Medium: Online Resource
    ISSN: 2326-5191 , 2326-5205
    URL: Issue
    URL: Article
    DOI: 10.1002/art.v68.7
    DOI: 10.1002/art.39652
    RVK:
    XA 10000
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2016
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