Early diagnostics of cardiac autonomic dysfunction in patients with systemic sclerosis

Aim. To study the potential of early diagnostics of cardiac autonomic dysfunction (CAD) in patients with systemic sclerosis (SSc).

Material and methods. The case-control study included 41 patients with SSc and 19 healthy individuals. Clinical tests (deep breathing test, blood pressure and heart rate (30:15) response to the orthostatic test) were performed, and patients were surveyed using the Composite Autonomic Symptom Score-31 (COMPASS-31) questionnaire.

Results. The total COMPASS-31 score in patients with SSc was 24,6 [13,6; 37,6], in the control group — 13,8 [2,7; 32,8] (p=0,021). The combination of vasomotor and orthostatic disorders was observed more often in patients with SSc than in the control group — in 23 (56,1%) and 3 (15,8%) participants, respectively (OR — odds ratio) =6,82; 95% confidence interval (CI): 1,72-27,06; p=0,003. In patients with SSc, the 30:15 ratio (heart rate response to orthostatic test) was significantly lower than in the control group (p=0,001); pathological test results indicating CAD were more often observed in patients with SSc (OR=6,50; 95% CI: 1,26-33,58; p=0,016). A positive correlation was found between the total COMPASS-31 score and the duration of SSc from the disease onset, diagnosis, and the duration of Raynaud's syndrome (R=0,39, R=0,35, R=0,38, p<0,05).

Conclusion. Patients with SSc show following early signs of CAD: vasomotor and orthostatic disorders are recorded 6,8 times more often according to the COMPASS-31 questionnaire, and pathological results of the 30:15 clinical test are detected 6,5 times more often.

1. Russian clinical guidelines. Rheumatology. Nasonov EL, ed. Moscow: GEOTAR-Media, 2017. p. 464 (In Russ.) ISBN: 978-5-9704-4261-6.

2. Bergamasco A, Hartmann N, Wallace L, et al. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol. 2019;11:257-73. doi:10.2147/CLEP.S191418.

3. Elhai M, Meune C, Avouac J, et al. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2012;51(6):1017-26. doi:10.1093/rheumatology/ker269.

4. Mani P, Gonzalez D, Chatterjee S, et al. Cardiovascular com­plications of systemic sclerosis: What to look for. Cleve Clin J Med. 2019;86(10):685-95. doi:10.3949/ccjm.86a.18109.

5. Ruzhentsova UYu. Microcirculatory disorders in scleroderma systematica: an association with vascular wall stiffness. Rheumatology Science and Practice. 2013;51(3):275-8. (In Russ.) doi:10.14412/1995-4484-2013-1501.

6. Di Battista M, Wasson CW, Alcacer-Pitarch B, et al. Autonomic dys­function in systemic sclerosis: A scoping review. Semin Arthritis Rheum. 2023;63:152268. doi:10.1016/j.semarthrit.2023.152268.

7. Cozzolino D, Naclerio C, Iengo R, et al. Cardiac autonomic dysfunction precedes the development of fibrosis in patients with systemic sclerosis. Rheumatology (Oxford). 2002;41(5):586-8. doi:10.1093/rheumatology/41.5.586.

8. Balbir-Gurman A, Shataylo V, Braun-Moscovici Y. Autoimmune Di­seases in Systemic Sclerosis Patients and Their Relatives: Data from a Single Center. Isr Med Assoc J. 2019;21(1):29-34.

9. Behr E, Ensam B. New approaches to predicting the risk of sudden death. Clin Med (Lond). 2016;16(3):283. doi:10.7861/clinmedicine.16-3-283.

10. Kobalava ZhD, Konradi AO, Nedogoda SV, et al. 2024 Clinical practice guidelines for Hypertension in adults. Russian Journal of Cardiology. 2024;29(9):6117. (In Russ.) doi:10.15829/1560-4071-2024-6117.

11. Sletten D, Suarez G, Low P, et al. COMPASS 31: a refined and abbreviated Composite Autonomic Symptom Score. Mayo Clin Proc. 2012;87(12):1196-201. doi:10.1016/j.mayocp.2012.10.013.

12. Dedov I, Shestakova M, Mayorov A, et al. Standards of Specialized Diabetes Care. Edited by Dedov II, Shestakova MV, Mayorov AYu. 11th Edition. Diabetes mellitus. 2023;26(2S):1-157. (In Russ.) doi:10.14341/DM13042.

13. Anichkov DA, Platonova AA. Clinical significance of heart rate variability indexes derived from 5-minute and 24-hour ECG recordings in patients with rheumatoid arthritis. Rational Pharmacotherapy in Cardiology. 2009;5(1):77-82. (In Russ.) doi:10.20996/1819-6446-2009-5-1-77-82.

14. Adler BL, Russell JW, Hummers LK, et al. Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed by the COMPASS-31 Questionnaire. J Rheumatol. 2018;45(8):1145-52. doi:10.3899/jrheum.170868.

15. Ostojic P, Atanaskovic M, Vujovic M, et al. SAT0336. Symptoms of autonomic dysfunction in patients with systemic sclerosis. Ann Rheum Dis 2020;79:1114. doi:10.1136/annrheumdis-2020-eular.4792.

16. Nussinovitch U, Gendelman O, Rubin S, et al. Autonomic Nervous System Indices in Patients with Systemic Sclerosis without Overt Cardiac Disease. Isr Med Assoc J. 2021;23(10):651-6.

17. Masini F, Galiero R, Pafundi PC, et al. Autonomic nervous system dysfunction correlates with microvascular damage in systemic sclerosis patients. J Scleroderma Relat Disord. 2021;6(3):256-63. doi:10.1177/23971983211020617.

18. Bajocchi G, Terlizzi R, Zanigni S, et al. Evidence of a selective nociceptive impairment in systemic sclerosis. Clin Exp Rheumatol. 2009;27(3 Suppl 54):9-14.

19. Gehad GM, Alshaimaa SK, May MF, et al. Heart rate variability stands up to bedside tests and composite autonomic symptom score-31 (COMPASS-31) questionnaire: Diagnostic tools for autonomic dysfunction in systemic sclerosis patients. Egypt Rheumatol. 2025;47(1):41-5. doi:10.1016/j.ejr.2024.11.004.

20. Lazareva NV, Kozlova LK. Clinical significance of the assessment of heart rate variability and QT interval dispersion in patients with systemic scleroderma. Ural Medical Journal. 2010;7(72):85-90. (In Russ.)