Pulmonary arterial hypertension in patients with systemic lupus erythematosus is a rare manifestation of a rare disease

The aim of the study was to determine the frequency, assess survival, and characterize the clinical features of systemic lupus erythematosus (SLE) in association with pulmonary arterial hypertension (PAH).

Material and methods. According to the results of echocardiographic screening, 8 patients with PAH-SLE were identified after verification of the diagnosis on a right heart catheterization. Two cohorts of patients with SLE observed at the V.A. Nasonova Research Institute of Rheumatology were taken as a comparison group: the first is historical, with a long follow-up period (n=154), the second is modern, with a short duration of the disease (n=400). The analyzed patient groups were examined according to a single scheme, differences in signs and survival were assessed. The intrahospital frequency of PAH in SLE was estimated based on statistical data for the period from 2008 to 2017.

Results. Despite the small number of patients in the main group, signs associated with the presence of PAH were identified: predominantly chronic course of SLE, with a long period (6–11 years) the course of the disease before the first clinical signs of PAH, the presence of Raynaud’s syndrome without capillaroscopic changes, Roand Smithautoantibodies. The survival rate of patients with PAH-SLE and the long-term observed SLE cohort did not differ. The in-hospital prevalence of SLE with echocardiographic screening was 0.29%.

Conclusion. PAH is a rare manifestation of SLE associated with the features of the course and clinical picture, which does not significantly affect survival when using PAH-specific therapy.

1. Volkov AV, Martynyuk TV. Pulmonary arterial hypertension in systemic connective tissue diseases: The current state of the problem. Nauchno-Prakticheskaya Revmatologia = Rheumatology Science and Practice. 2018; 56(4):474-485 (In Russ.). doi: 10.14412/1995-4484-2018-474-485

2. Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: Identifying systemic sclerosis as a unique phenotype. Chest. 2010;138(6):1383-1394. doi: 10.1378/chest.10-0260

3. Distler O, Ofner C, Huscher D, Jordan S, Ulrich S, Stähler G, et al. Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis. Rheumatology (Oxford). 2024;63(4):1139-1146. doi: 10.1093/rheumatology/kead360

4. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110-2121. doi: 10.1056/NEJMra1100359

5. Kaul A, Gordon C, Crow MK, Touma Z, Urowitz MB, van Vollenhoven R, et al. Systemic lupus erythematosus. Nat Rev Dis Primers. 2016;2:16039. doi: 10.1038/nrdp.2016.39

6. Norman RA. The history of lupus erythematosus and discoid lupus: From Hippocrates to the present. Lupus Open Access. 2016;1(1):102. doi: 10.35248/2684-1630.16.1.102

7. Lv TT, Wang P, Guan SY, Li HM, Li XM, Wang B, et al. Prevalence of pulmonary hypertension in systemic lupus erythematosus: A meta-analysis. Ir J Med Sci. 2018;187(3):723-730. doi: 10.1007/s11845-017-1727-4

8. Yang X, Mardekian J, Sanders KN, Mychaskiw MA, Thomas J. Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: A systematic review of the literature. Clin Rheumatol. 2013;32:1519-1531.

9. Pan TL, Thumboo J, Boey ML. Primary and secondary pulmonary hypertension in systemic lupus erythematosus. Lupus. 2000;9:338-342.

10. Pérez-Peñate GM, Rúa-Figueroa I, Juliá-Serdá G, León-Marrero F, García-Quintana A, Ortega-Trujillo JR, et al. Pulmonary arterial hypertension in systemic lupus erythematosus: Prevalence and predictors. J Rheumatol. 2016;43(2):323-329. doi: 10.3899/jrheum.150451

11. Ruiz-Irastorza G, Garmendia M, Villar I, Egurbide MV, Aguirre C. Pulmonary hypertension in systemic lupus erythematosus: Prevalence, predictors and diagnostic strategy. Autoimmun Rev. 2013;12(3):410-415. doi: 10.1016/j.autrev.2012.07.010

12. Marasco E, Düsing C, Keymel S, Bortoluzzi A, Bracaglia C, Canuet M, et al. Pulmonary arterial hypertension in systemic lupus erythematosus: Identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort. Lupus Sci Med. 2025;12(1):e001471. doi: 10.1136/lupus-2024-001471

13. Lian F, Chen D, Wang Y, Ye Y, Wang X, Zhan Z, et al. Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus. Rheumatol Int. 2012;32(6):1727-1731. doi: 10.1007/s00296-011-1880-4

14. Kasparian A, Floros A, Gialafos E, Kanakis M, Tassiopoulos S, Kafasi N, et al. Raynaud’s phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus. Lupus. 2007;16(7):505-508. doi: 10.1177/0961203307080629

15. Tani C, Elefante E, Arnaud L, Barreira SC, Bulina I, Cavagna L, et al. Rare clinical manifestations in systemic lupus erythematosus: A review on frequency and clinical presentation. Clin Exp Rheumatol. 2022;40(Suppl 134(5)):93-102. doi: 10.55563/clinexprheumatol/jrz47c

16. Arnaud L, Ruiz-Irastorza G, Aranow C, Bernatsky S, Dall’Era M, Adelowo O, et al.; European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal, Diseases; Systemic Lupus Erythematosus International Collaborating Clinics group; European Lupus Society rare systemic lupus erythematosus taskforce member panel. ERN ReCONNET-SLICC-SLEuroexpert consensus on the therapeutic management of rare systemic lupus erythematosus manifestations. Lancet Rheumatol. 2025;7(7):e505-e518. doi: 10.1016/S2665-9913(25)00063-3

17. Chazova IE, Avdeev SN, Arutyunov GP, Volkov AV, Martynyuk TV, Nakonechnikov SN, et al. Diagnosis and treatment of pulmonary hypertension. Russian recommendations. Cardiovascular Therapy and Prevention. 2007;6(6 S2):1-20 (In Russ.).

18. Chazova IE, Avdeev SN, Tsareva NA, Volkov AV, Martyniuk TV, Nakonechnikov SN. Clinical guidelines for the diagnosis and treatment of pulmonary hypertension. Terapevticheskii arkhiv. 2014;86(9):4-23 (In Russ.).

19. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40(9):1725. doi: 10.1002/art.1780400928

20. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271-1277. doi: 10.1002/art.1780251101

21. Asif S, Rasheed A, Mahmud TE, Asghar A. Frequency and predictors of pulmonary hypertension in patients with systemic lupus erythematosus. Pak J Med Sci. 2019;35(1):86-89. doi: 10.12669/pjms.35.1.405

22. Atsumi T, Bae SC, Gu H, Huang WN, Li M, Nikpour M, et al. Risk factors for pulmonary arterial hypertension in patients with systemic lupus erythematosus: A systematic review and expert consensus. ACR Open Rheumatol. 2023;5(12):663-676. doi: 10.1002/acr2.11611

23. Liu Y, Cheng Z, Zha B, Chen X, Gong Z, Ji L, et al. Risk factors of pulmonary arterial hypertension in patients with systemic lupus erythematosus: A meta-analysis. Lupus. 2023;32(11):1310-1319. doi: 10.1177/09612033231202398

24. Hachulla E, Jais X, Cinquetti G, Clerson P, Rottat L, Launay D, et al.; French Collaborators Recruiting Members(*). Pulmonary arterial hypertension associated with systemic lupus erythematosus: Results from the French Pulmonary Hypertension Registry. Chest. 2018;153(1):143-151. doi: 10.1016/j.chest.2017.08.014

25. Riancho-Zarrabeitia L, Martínez-Taboada V, Rúa-Figueroa I, Alonso F, Galindo-Izquierdo M, Ovalles J, et al. Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) implies a more severe disease with more damage accrual and higher mortality. Lupus. 2020;29(12):1556-1565. doi: 10.1177/0961203320950477

26. Chi E, Chandrasekara S, Keating DT. Severe pulmonary arterial hypertension and cardiogenic shock in acute systemic lupus erythematosus. BMJ Case Rep. 2025;18(1):e262651. doi: 10.1136/bcr2024-262651

27. Luppino-Assad AP, Alves Junior JL, Figueiredo Neves Yuki E, Seguro LPC, Pasoto SG, Fernandes CJCDS, et al. Reversibility of pulmonary hypertension in systemic lupus erythematosus after induction immunosuppressive therapy: An inflammatory manifestation? Lupus. 2025;34(1):18-27. doi: 10.1177/09612033241301183

28. Zen M, Salmaso L, Barbiellini Amidei C, Fedeli U, Bellio S, Iaccarino L, et al. Mortality and causes of death in systemic lupus erythematosus over the last decade: Data from a large populationbased study. Eur J Intern Med. 2023;112:45-51. doi: 10.1016/j.ejim.2023.02.004

29. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al.; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731. doi: 10.1093/eurheartj/ehac237

30. Avdeev SN, Barbarash OL, Valieva ZS, Volkov AV, Veselova TN, Galyavich AS, et al. 2024 clinical practice guidelines for pulmonary hypertension, including chronic thromboembolic pulmonary hypertension. Russian Journal of Cardiology. 2024;29(11):6161 (In Russ.). doi: 10.15829/1560-4071-2024-6161