Updated international recommendations (EULAR 2023) for the treatment of systemic sclerosis
https://doi.org/10.14412/1996-7012-2026-2-21-28
Abstract
In recent years, significant progress has been achieved in the study of systemic sclerosis (SSc). The treatment of SSc includes conventional synthetic disease-modifying antirheumatic drugs, biologic agents, including anti-B-cell drugs and interleukin-6 inhibitors, as well as antifibrotic agents. Since the last publication of the EULAR recommendations for the treatment of SSc in 2017, changes have occurred; therefore, an update was prepared in which the treatment of the full spectrum of SSc manifestations is considered more broadly. In the updated document 22 recommendations were formulated, and 8 therapeutic domains were identified. The article presents the EULAR 2023 recommendations for the treatment of SSc, as well as promising directions for scientific research.
About the Authors
A. S. TrofimovaRussian Federation
Anna Sergeevna Trofimova
41, Kirochnaya Street, Saint Petersburg 195015
V. I. Mazurov
Russian Federation
41, Kirochnaya Street, Saint Petersburg 195015
V. Yu. Myachikova
Russian Federation
2, Akkuratova Street, Saint Petersburg 197341
E. S. Kuvardin
Russian Federation
2, Akkuratova Street, Saint Petersburg 197341
A. L. Maslyanskiy
Russian Federation
2, Akkuratova Street, Saint Petersburg 197341
34A, Kashirskoe Shosse, Moscow 115522
E. A. Trofimov
Russian Federation
41, Kirochnaya Street, Saint Petersburg 195015
References
1. Guseva NG. Systemic sclerosis: early diagnosis and prognosis. Nauchno-prakticheskaya revmatologiya. 2007;45(1):39-45. (In Russ.).
2. Lescoat A, Huang S, Carreira PE, et al. Cutaneous manifestations, clinical characteristics, and prognosis of patients with systemic sclerosis sine scleroderma: data from the international EUSTAR database. JAMA Dermatol. 2023 Aug;159(8):837-47. doi: 10.1001/jamadermatol.2023.1729.
3. Trofimova AS, Shokhin AA, Trofimov EA, Shostak MS. Clinical case of systemic sclerosis in a male patient: gender differences and possibilities of capillaroscopy. Modern Rheumatology Journal. 2024;18(1):84-9. (In Russ.). doi: 10.14412/1996-7012-2024-1-84-89.
4. Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76(8):1327-39. doi: 10.1136/annrheumdis-2016-209909.
5. Del Galdo F, Lescoat A, Conaghan PG, et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Ann Rheum Dis. 2025 Jan;84(1):29-40. doi: 10.1136/ard-2024-226430.
6. Roustit M, Blaise S, Allanore Y, et al. Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud’s phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis. 2013 Oct; 72(10):1696-9. doi:10.1136/annrheumdis-2012-202836.
7. Kuwana M, Blair C, Takahashi T, et al. Initial combination therapy of ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) in the modified intention-totreat population of the AMBITION study: post hoc analysis. Ann Rheum Dis. 2020 May; 79(5):626-34. doi: 10.1136/annrheumdis-2019-216274.
8. Gaine S, Chin K, Coghlan G, et al. Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. Eur Respir J. 2017 Aug;50(2): 1602493. doi: 10.1183/13993003.02493-2016.
9. Humbert M, Coghlan JG, Ghofrani HA, et al. Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2. Ann Rheum Dis. 2017 Feb;76(2):422-6. doi: 10.1136/annrheumdis-2015-209087.
10. Khan MS, Usman MS, Siddiqi TJ, et al. Is anticoagulation beneficial in pulmonary arterial hypertension? Circ Cardiovasc Qual Outcomes. 2018 Sep;11(9):e004757. doi: 10.1161/CIRCOUTCOMES.118.004757.
11. Petcu A, Ghib LJ, Grad SM, et al. Upper gastrointestinal involvement in systemic sclerosis: Findings in a real-life setting. Exp Ther Med. 2019 Dec;18(6):5095-5100. doi: 10.3892/etm.2019.8125.
12. Stern EK, Carlson DA, Falmagne S, et al. Abnormal esophageal acid exposure on highdose proton pump inhibitor therapy is common in systemic sclerosis patients. Neurogastroenterol Motil. 2018 Feb;30(2): 10.1111/nmo.13247. doi:10.1111/nmo.13247.
13. Foocharoen C, Chunlertrith K, Mairiang P, et al. Effectiveness of add-on therapy with domperidone vs alginic acid in proton pump inhibitor partial response gastro-oesophageal reflux disease in systemic sclerosis: randomized placebo-controlled trial. Rheumatology (Oxford). 2017 Feb;56(2):214- 22. doi: 10.1093/rheumatology/kew216.
14. Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016 Sep;4(9):708-19. doi: 10.1016/S2213-2600(16)30152-7.
15. Ebata S, Yoshizaki A, Oba K, et al. Safety and efficacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-initiated, randomised, placebo-controlled trial. Lancet Rheumatol. 2021 Jul;3(7):e489-e497. doi: 10.1016/S2665-9913(21)00107-7.
16. Khanna D, Denton CP, Jahreis A, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet. 2016 Jun;387(10038): 2630-40. doi: 10.1016/S0140-6736(16)00232-4.
17. Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020 Oct; 8(10):963-74. doi: 10.1016/S2213-2600(20)30318-0.
18. Maher TM, Tudor VA, Saunders P, et al. Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL): a double-blind, doubledummy, randomised, controlled, phase 2b trial. Lancet Respir Med. 2023 Jan;11(1):45-54. doi: 10.1016/S2213-2600(22)00359-9.
19. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019 Jun;380(26):2518-28. doi: 10.1056/NEJMoa1903076.
20. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019 Oct; 381(18):1718-27. doi: 10.1056/NEJMoa1908681.
21. Sullivan KM, Goldmuntz EA, Keyes-Elstein L, et al. Myeloablative autologous stemcell transplantation for severe scleroderma. N Engl J Med. 2018 Jan ;378(1):35-47. doi: 10.1056/nejmoa1703327.
Review
For citations:
Trofimova AS, Mazurov VI, Myachikova VY, Kuvardin ES, Maslyanskiy AL, Trofimov EA. Updated international recommendations (EULAR 2023) for the treatment of systemic sclerosis. Sovremennaya Revmatologiya=Modern Rheumatology Journal. 2026;20(2):21-28. (In Russ.) https://doi.org/10.14412/1996-7012-2026-2-21-28
JATS XML




































