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)...
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BMJ Publishing Group
2025-06-01
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| Series: | Lupus Science and Medicine |
| Online Access: | https://lupus.bmj.com/content/12/1/e001471.full |
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| author | Carlo Alberto Scirè Alessandra Bortoluzzi Micaela Fredi Matthias Schneider Veronica Codullo Carlomaurizio Montecucco Alain Meyer Ilaria Cavazzana Lorenzo Cavagna Franco Franceschini Andreas Schwarting Konstantinos Triantafyllias Gamal Chehab Christina Düsing Jutta Richter Ettore Silvagni Oliver Sander Giovanni Zanframundo Claudia Bracaglia Matthieu Canuet Stefano Ghio Rebecca Fischer Emiliano Marasco Sezgin Sahin Lisa Keller Marianne Riou Stefanie Keymel |
| author_facet | Carlo Alberto Scirè Alessandra Bortoluzzi Micaela Fredi Matthias Schneider Veronica Codullo Carlomaurizio Montecucco Alain Meyer Ilaria Cavazzana Lorenzo Cavagna Franco Franceschini Andreas Schwarting Konstantinos Triantafyllias Gamal Chehab Christina Düsing Jutta Richter Ettore Silvagni Oliver Sander Giovanni Zanframundo Claudia Bracaglia Matthieu Canuet Stefano Ghio Rebecca Fischer Emiliano Marasco Sezgin Sahin Lisa Keller Marianne Riou Stefanie Keymel |
| author_sort | Carlo Alberto Scirè |
| collection | DOAJ |
| description | 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. |
| format | Article |
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| institution | DOAJ |
| issn | 2053-8790 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | Lupus Science and Medicine |
| spelling | doaj-art-2f78d9d8db04417aab0bd3d7d8a1e7b92025-08-20T02:39:52ZengBMJ Publishing GroupLupus Science and Medicine2053-87902025-06-0112110.1136/lupus-2024-001471Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohortCarlo Alberto Scirè0Alessandra Bortoluzzi1Micaela Fredi2Matthias Schneider3Veronica Codullo4Carlomaurizio Montecucco5Alain Meyer6Ilaria Cavazzana7Lorenzo Cavagna8Franco Franceschini9Andreas Schwarting10Konstantinos Triantafyllias11Gamal Chehab12Christina Düsing13Jutta Richter14Ettore Silvagni15Oliver Sander16Giovanni Zanframundo17Claudia Bracaglia18Matthieu Canuet19Stefano Ghio20Rebecca Fischer21Emiliano Marasco22Sezgin Sahin23Lisa Keller24Marianne Riou25Stefanie Keymel26Division of Rheumatology, University of Milan-Bicocca, Milano, ItalySection of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, ItalyRheumatology and Clinical Immunology Unit, ASST Spedali Civili and Clinical and Experimental Science Department, University of Brescia, Brescia, ItalyDepartment for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, GermanyDivision of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDivision of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, ItalyRheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Strasbourg University Hospitals, Strasbourg, FranceRheumatology and Clinical Immunology Unit, ASST Spedali Civili and Clinical and Experimental Science Department, University of Brescia, Brescia, ItalyDivision of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, ItalyRheumatology and Clinical Immunology Unit, ASST Spedali Civili and Clinical and Experimental Science Department, University of Brescia, Brescia, ItalyRheumatology, Rheumatology Center Rhineland-Palatinate, Bad Kreuznach, GermanyDivision of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, GermanyRheumatology, Heinrich Heine University Düsseldorf, Dusseldorf, GermanyDepartment for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, GermanyDepartment for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, GermanySection of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, ItalyDepartment for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, GermanyDivision of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, ItalyDivision of Rheumatology, Bambino Gesu Pediatric Hospital IRCCS, Roma, ItalyService de Pneumologie Centre de Compétence de l’Hypertension Artérielle Pulmonaire, Les Hôpitaux Universitaires de Strasbourg, Strasbourg, FranceDivision of Cardiology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDepartment for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, GermanyDivision of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, ItalyDepartment of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, TurkeyDivision of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, GermanyService de Pneumologie Centre de Compétence de l’Hypertension Artérielle Pulmonaire, Les Hôpitaux Universitaires de Strasbourg, Strasbourg, FranceDepartment of Cardiology, Pneumology and Angiology, Heinrich-Heine-Universitat Dusseldorf, Dusseldorf, GermanyObjectives 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.https://lupus.bmj.com/content/12/1/e001471.full |
| spellingShingle | Carlo Alberto Scirè Alessandra Bortoluzzi Micaela Fredi Matthias Schneider Veronica Codullo Carlomaurizio Montecucco Alain Meyer Ilaria Cavazzana Lorenzo Cavagna Franco Franceschini Andreas Schwarting Konstantinos Triantafyllias Gamal Chehab Christina Düsing Jutta Richter Ettore Silvagni Oliver Sander Giovanni Zanframundo Claudia Bracaglia Matthieu Canuet Stefano Ghio Rebecca Fischer Emiliano Marasco Sezgin Sahin Lisa Keller Marianne Riou Stefanie Keymel Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort Lupus Science and Medicine |
| title | Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort |
| title_full | Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort |
| title_fullStr | Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort |
| title_full_unstemmed | Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort |
| title_short | Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort |
| title_sort | pulmonary arterial hypertension in systemic lupus erythematosus identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort |
| url | https://lupus.bmj.com/content/12/1/e001471.full |
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