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  • P39 Analysis of nailfold capillaroscopy findings and clinical features of patients with systemic lupus erythematosus and pulmonary arterial hypertension

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P39 Analysis of nailfold capillaroscopy findings and clinical features of patients with systemic lupus erythematosus and pulmonary arterial hypertension
  1. Emiliano Marasco1,
  2. Christina Duesing2,
  3. Stephanie Keymel3,
  4. Giovanni Zanframundo4,
  5. Veronica Codullo4,
  6. Oliver Sander2,
  7. Gamal Chehab2,
  8. Jutta Richter2,
  9. Rebecca Fischer-Betz2,
  10. Claudia Bracaglia5,
  11. Sezgin Sahin6,
  12. Konstantinos Triantafyllias7,
  13. Andreas Schwarting7,
  14. Lisa Keller8,
  15. Alain Meyer9,
  16. Matthieu Canuet10,
  17. Marianne Riou10,
  18. Ilaria Cavazzana11,
  19. Micaela Fredi11,
  20. Franco Franceschini11,
  21. Ettore Silvagni12,
  22. Alessandra Bortoluzzi12,
  23. Carlo Alberto Scirè12,
  24. Carlomaurizio Montecucco4,
  25. Lorenzo Cavagna4 and
  26. Matthias Schneider2
  1. 1Bambino Gesu’ Children Hospital, Rheumatology, Rome, Italy
  2. 2Heinrich-Heine-University Duesseldorf, Policlinic and Hiller Research Unit for Rheumatology, Duesseldorf, Germany
  3. 3University Hospital Duesseldorf, Dept. of Cardiology, Pneumology and Angiology, Duesseldorf, Germany
  4. 4Fondazione IRCCS Policlinico San Matteo, Division of Rheumatology, Pavia, Italy
  5. 5Bambino Gesu’ Children Hospital, Unit of Rheumatology, Rome, Italy
  6. 6Istanbul University Cerrahpasa, Dept. of Pediatric Rheumatology, İstanbul,Turkey
  7. 7ACURA Rheumatology Center Rhineland-Palatinate, Rheumatology, Bad Kreuznach, Germany
  8. 8University Hospital of Mainz, Division of Rheumatology, Mainz, Germany
  9. 9Hôpitaux Universitaires de Strasbourg, Rheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Strasbourg, France
  10. 10Hôpitaux Universitaires de Strasbourg, Service de Pneumologie Centre de Compétence de l’Hypertension Artérielle Pulmonaire, Strasbourg, France
  11. 11ASST Spedali Civili and Clinical and Experimental Science Dept., University of Brescia, Rheumatology and Clinical Immunology Unit, Brescia, Italy
  12. 12University of Ferrara, Section of Rheumatology, Dept. of Medical Sciences, Ferrara, Italy

Abstract

Objective The aim of our work is to analyze the clinical and demographic features and nailfold capillary changes in patients with SLE-related PAH compared to a group of SLE patients without PAH.

Methods We identified and selected 20 patients with SLE and type I PAH and collected demographic, clinical and laboratory features from 8 rheumatology centers across Europe. We could perform NVC on 9 patients. We selected as controls 68 patients with SLE who underwent cardiopulmonary screening to exclude PAH: we collected demographic, clinical and laboratory features and performed NVC. The presence of SD pattern was assessed according to Smith et al (Autoimmunity Reviews 2019). Patients satisfied the 2019 EULAR/ACR SLE classification criteria. We excluded patients with a diagnosis of mixed tissue disease and overlap syndrome.

Results All patients with SLE-PAH were female; age and disease duration were not different from SLE patients without PAH. LAC+ and anti-RNP+ was more prevalent in patients with SLE-PAH. No differences were observed for anti-Sm, anti-Ro, anti-La and anti-phospholipid antibodies. Of clinical features, skin and CNS involvement were more prevalent in patients with SLE-PAH than in SLE controls. Raynaud’s phenomenon was more prevalent in patients with SLE-PAH than in SLE controls. In patients with SLE-PAH we observed a significantly higher prevalence of scleroderma pattern at NVC than in SLE controls: patients with SLE-PAH showed a lower number of capillary density and a higher frequency of megacapillaries. In multivariate analysis, Raynaud phenomenon and anti-RNP are predictors of PAH in patients with SLE. The McFadden’s R-squared for the model is 0.30.

Conclusions Our data show that LAC+, RNP+, Raynaud’s, Skin and CNS involvement and a SD pattern at NVC is more prevalent in patients with SLE PAH than in patients with SLE without PAH. Our results point to a generalized 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 with a high risk of developing PAH.

Acknowledgements The study was supported by SLEuro.

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ .

https://doi.org/10.1136/lupus-2024-el.93

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