系统性红斑狼疮肺动脉高压：多中心回顾性队列中危险因素和血流动力学特征的识别。

IF 3.7 2区医学 Q1 RHEUMATOLOGY

Lupus Science & Medicine Pub Date : 2025-06-12 DOI:10.1136/lupus-2024-001471

Emiliano Marasco, Christina Düsing, Stefanie Keymel, Alessandra Bortoluzzi, Claudia Bracaglia, Matthieu Canuet, Ilaria Cavazzana, Gamal Chehab, Veronica Codullo, Rebecca Fischer, Franco Franceschini, Micaela Fredi, Stefano Ghio, Lisa Keller, Alain Meyer, Carlomaurizio Montecucco, Jutta Richter, Marianne Riou, Sezgin Sahin, Oliver Sander, Andreas Schwarting, Carlo Alberto Scirè, Ettore Silvagni, Konstantinos Triantafyllias, Giovanni Zanframundo, Lorenzo Cavagna, Matthias Schneider

{"title":"系统性红斑狼疮肺动脉高压：多中心回顾性队列中危险因素和血流动力学特征的识别。","authors":"Emiliano Marasco, Christina Düsing, Stefanie Keymel, Alessandra Bortoluzzi, Claudia Bracaglia, Matthieu Canuet, Ilaria Cavazzana, Gamal Chehab, Veronica Codullo, Rebecca Fischer, Franco Franceschini, Micaela Fredi, Stefano Ghio, Lisa Keller, Alain Meyer, Carlomaurizio Montecucco, Jutta Richter, Marianne Riou, Sezgin Sahin, Oliver Sander, Andreas Schwarting, Carlo Alberto Scirè, Ettore Silvagni, Konstantinos Triantafyllias, Giovanni Zanframundo, Lorenzo Cavagna, Matthias Schneider","doi":"10.1136/lupus-2024-001471","DOIUrl":null,"url":null,"abstract":"Objectives: The aim of our work was to identify specific patterns in clinical features and nailfold capillary changes that may help in screening for pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).Methods: We identified patients with SLE and type I PAH (n=20) without other connective tissue diseases and collected demographic, clinical and laboratory features. We selected as controls patients with SLE who underwent cardiopulmonary screening to exclude PAH (n=87): we collected demographic, clinical and laboratory features and performed nailfold videocapillaroscopy (NVC).Results: All patients with SLE-PAH were women; age and disease duration were not different from patients with SLE without PAH. Lupus anticoagulant (LAC)+and anti-ribonucleoprotein (RNP)+were more prevalent in patients with SLE-PAH (respectively, PAH 45.0% vs no-PAH 20.5%, p=0.042; PAH 45.0% vs no-PAH 19.5%, p=0.035). No differences were observed for anti-Sm, anti-Ro, anti-La and anti-cardiolipin and anti-beta2GPI antibodies. Among clinical features, mucocutaneous and central nervous system involvement were more prevalent in patients with SLE-PAH than in SLE controls (respectively, PAH 65.0% vs no-PAH 34.5%, p=0.024; PAH 25.0% vs no-PAH 8.0%, p=0.046). Raynaud's phenomenon (RP) was more prevalent in patients with SLE-PAH than in SLE controls (PAH 60.0% vs no-PAH 13.8%, p<0.001). RP was a predictor of PAH in patients with SLE (OR 3.8 (0.9-14.8)). We performed NVC on nine patients with PAH and on controls: we observed a significantly higher prevalence of scleroderma pattern at NVC in SLE-PAH than controls (PAH 66.7% vs no-PAH 9.2%, p<0.001). Patients with SLE-PAH showed a lower number of capillary density and a higher frequency of giant capillaries.Conclusions: Our data showed that LAC+, RNP+, RP and a scleroderma pattern at NVC was indicative for patients with SLE-PAH. Our results pointed to generalised microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE at risk of developing PAH.","PeriodicalId":18126,"journal":{"name":"Lupus Science & Medicine","volume":"12 1","pages":""},"PeriodicalIF":3.7000,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164622/pdf/","citationCount":"0","resultStr":"{\"title\":\"Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort.\",\"authors\":\"Emiliano Marasco, Christina Düsing, Stefanie Keymel, Alessandra Bortoluzzi, Claudia Bracaglia, Matthieu Canuet, Ilaria Cavazzana, Gamal Chehab, Veronica Codullo, Rebecca Fischer, Franco Franceschini, Micaela Fredi, Stefano Ghio, Lisa Keller, Alain Meyer, Carlomaurizio Montecucco, Jutta Richter, Marianne Riou, Sezgin Sahin, Oliver Sander, Andreas Schwarting, Carlo Alberto Scirè, Ettore Silvagni, Konstantinos Triantafyllias, Giovanni Zanframundo, Lorenzo Cavagna, Matthias Schneider\",\"doi\":\"10.1136/lupus-2024-001471\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Objectives: The aim of our work was to identify specific patterns in clinical features and nailfold capillary changes that may help in screening for pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).Methods: We identified patients with SLE and type I PAH (n=20) without other connective tissue diseases and collected demographic, clinical and laboratory features. We selected as controls patients with SLE who underwent cardiopulmonary screening to exclude PAH (n=87): we collected demographic, clinical and laboratory features and performed nailfold videocapillaroscopy (NVC).Results: All patients with SLE-PAH were women; age and disease duration were not different from patients with SLE without PAH. Lupus anticoagulant (LAC)+and anti-ribonucleoprotein (RNP)+were more prevalent in patients with SLE-PAH (respectively, PAH 45.0% vs no-PAH 20.5%, p=0.042; PAH 45.0% vs no-PAH 19.5%, p=0.035). No differences were observed for anti-Sm, anti-Ro, anti-La and anti-cardiolipin and anti-beta2GPI antibodies. Among clinical features, mucocutaneous and central nervous system involvement were more prevalent in patients with SLE-PAH than in SLE controls (respectively, PAH 65.0% vs no-PAH 34.5%, p=0.024; PAH 25.0% vs no-PAH 8.0%, p=0.046). Raynaud's phenomenon (RP) was more prevalent in patients with SLE-PAH than in SLE controls (PAH 60.0% vs no-PAH 13.8%, p<0.001). RP was a predictor of PAH in patients with SLE (OR 3.8 (0.9-14.8)). We performed NVC on nine patients with PAH and on controls: we observed a significantly higher prevalence of scleroderma pattern at NVC in SLE-PAH than controls (PAH 66.7% vs no-PAH 9.2%, p<0.001). Patients with SLE-PAH showed a lower number of capillary density and a higher frequency of giant capillaries.Conclusions: Our data showed that LAC+, RNP+, RP and a scleroderma pattern at NVC was indicative for patients with SLE-PAH. Our results pointed to generalised microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE at risk of developing PAH.\",\"PeriodicalId\":18126,\"journal\":{\"name\":\"Lupus Science & Medicine\",\"volume\":\"12 1\",\"pages\":\"\"},\"PeriodicalIF\":3.7000,\"publicationDate\":\"2025-06-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12164622/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Lupus Science & Medicine\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1136/lupus-2024-001471\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"RHEUMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Lupus Science & Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1136/lupus-2024-001471","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}

引用次数: 0

摘要

目的：我们工作的目的是确定临床特征和甲襞毛细血管变化的特定模式，这可能有助于筛查系统性红斑狼疮（SLE）患者的肺动脉高压（PAH）。方法：选取无其他结缔组织疾病的SLE和I型PAH患者（n=20），收集人口统计学、临床和实验室特征。我们选择了接受心肺筛查以排除PAH的SLE患者作为对照（n=87）：我们收集了人口统计学、临床和实验室特征，并进行了甲襞视频毛细血管镜检查（NVC）。结果：SLE-PAH患者均为女性；年龄和病程与无PAH的SLE患者无差异。狼疮抗凝剂(LAC)+和抗核蛋白(RNP)+在SLE-PAH患者中更为普遍(PAH 45.0% vs无PAH 20.5%, p=0.042；PAH 45.0% vs no-PAH 19.5%, p=0.035)。抗sm、抗ro、抗la、抗心磷脂和抗β 2gpi抗体无差异。在临床特征中，SLE-PAH患者的皮肤粘膜和中枢神经系统受累程度高于SLE对照组(分别为PAH 65.0% vs无PAH 34.5%, p=0.024；PAH 25.0% vs no-PAH 8.0%, p=0.046)。雷诺现象（RP）在SLE-PAH患者中比在SLE对照组中更为普遍（PAH 60.0% vs无PAH 13.8%）。结论：我们的数据显示，LAC+、RNP+、RP和NVC的硬皮病模式是SLE-PAH患者的指示性指标。我们的研究结果表明，SLE-PAH患者普遍存在微血管受累和高凝状态。我们确定的变量可用于实施筛选算法，以识别SLE患者发展为PAH的风险。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort.

Objectives: The aim of our work was to identify specific patterns in clinical features and nailfold capillary changes that may help in screening for pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).

Methods: We identified patients with SLE and type I PAH (n=20) without other connective tissue diseases and collected demographic, clinical and laboratory features. We selected as controls patients with SLE who underwent cardiopulmonary screening to exclude PAH (n=87): we collected demographic, clinical and laboratory features and performed nailfold videocapillaroscopy (NVC).

Results: All patients with SLE-PAH were women; age and disease duration were not different from patients with SLE without PAH. Lupus anticoagulant (LAC)+and anti-ribonucleoprotein (RNP)+were more prevalent in patients with SLE-PAH (respectively, PAH 45.0% vs no-PAH 20.5%, p=0.042; PAH 45.0% vs no-PAH 19.5%, p=0.035). No differences were observed for anti-Sm, anti-Ro, anti-La and anti-cardiolipin and anti-beta2GPI antibodies. Among clinical features, mucocutaneous and central nervous system involvement were more prevalent in patients with SLE-PAH than in SLE controls (respectively, PAH 65.0% vs no-PAH 34.5%, p=0.024; PAH 25.0% vs no-PAH 8.0%, p=0.046). Raynaud's phenomenon (RP) was more prevalent in patients with SLE-PAH than in SLE controls (PAH 60.0% vs no-PAH 13.8%, p<0.001). RP was a predictor of PAH in patients with SLE (OR 3.8 (0.9-14.8)). We performed NVC on nine patients with PAH and on controls: we observed a significantly higher prevalence of scleroderma pattern at NVC in SLE-PAH than controls (PAH 66.7% vs no-PAH 9.2%, p<0.001). Patients with SLE-PAH showed a lower number of capillary density and a higher frequency of giant capillaries.

Conclusions: Our data showed that LAC+, RNP+, RP and a scleroderma pattern at NVC was indicative for patients with SLE-PAH. Our results pointed to generalised microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE at risk of developing PAH.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Lupus Science & Medicine RHEUMATOLOGY-

CiteScore

5.30

自引率

7.70%

发文量

审稿时长

15 weeks

期刊介绍： Lupus Science & Medicine is a global, peer reviewed, open access online journal that provides a central point for publication of basic, clinical, translational, and epidemiological studies of all aspects of lupus and related diseases. It is the first lupus-specific open access journal in the world and was developed in response to the need for a barrier-free forum for publication of groundbreaking studies in lupus. The journal publishes research on lupus from fields including, but not limited to: rheumatology, dermatology, nephrology, immunology, pediatrics, cardiology, hepatology, pulmonology, obstetrics and gynecology, and psychiatry.