Clinical and Epidemiological Characteristics of Scleroderma in Children of the Samara Region: a Single-Stage Retrospective Study

Background. Juvenile scleroderma is an autoimmune disease of connective tissue in children of different ages. Currently, there is no structured information about the course of this disease in children of The Samara Region, which makes it difficult to regularly monitor and timely correct the treatment of the disease.

The aim of the study is to create a regional registry, as well as to conduct a clinical and epidemiological analysis of scleroderma in children in the Samara region for 2018–2023. Methods. A single-stage retrospective study was conducted in the period from 2018 to 2023, which included 75 patients treated at the V.P. Polyakov Samara Regional Clinical Cardiology Dispensary, in the Department of Pediatric Cardiac Surgery and Cardiorheumatology with a diagnosis of scleroderma.

Results. There has been a 1.3-fold increase in the prevalence of juvenile scleroderma among children (0–17 years old) over the period 2018–2023. In 13% of cases, patients were misdiagnosed, which is probably due to the diversity and non-specificity of the clinical picture of juvenile scleroderma. According to the results of the study, a long diagnostic search can be noted — it took more than two years for 17.3% to make a correct diagnosis, which is most likely due to the lack of an interdisciplinary approach to the management of juvenile scleroderma, as well as the late treatment of parents by a doctor. The complicated course of systemic scleroderma occurred in 1.3% of cases and was characterized by vascular disorders such as nail osteolysis of the distal phalanges, which was associated with an erroneous diagnosis at the outpatient stage and a delayed referral to a rheumatologist.

Conclusion. The conducted research allows us to conclude that it is advisable to create a local registry of children with scleroderma, the analysis of which will help pediatricians and rheumatologists of both outpatient and inpatient levels to respond in time to existing deviations in the diagnosis and therapy of scleroderma and optimize approaches to the management of patients with this profile. The limitation of the presented study is the small number of children (n = 75). It can also be assumed that the study did not include all children in the region suffering from scleroderma, given the lack of a registry, which makes it much more difficult to assess the course of the disease.

1. Detskaya revmatologiya: A guide for doctors. Baranov AA, Bazhenova LK, eds. Moscow: Medicine; 2002. pp. 138–178. (In Russ).

2. Sevostyanov VK, Lototslaya PS, Balashov SL, et al. Clinical and epidemiological analysis of juvenile systemic scleroderma according to the Moscow register of children with rheumatic diseases. Health Care Standardization Problems. 2022;(5-6):49–58. (In Russ). doi: https://doi.org/10.26347/1607-2502202201-02053-058]

3. Rossato M, Affandi AJ, Thordardottir S, et al. Association of MicroRNA-618 Expression with Altered Frequency and Activation of Plasmacytoid Dendritic Cells in Patients With Systemic Sclerosis. Arthritis Rheumatol. 2017;69(9):1891–1902. doi: https://doi.org/10.1002/art.40163

4. De Martinis M, Ciccarelli F, Sirufo MM, Ginaldi L. An overview of environmental risk factors in systemic sclerosis. Expert Rev Clin Immunol. 2016;12(4):465–478. doi: https://doi.org/10.1586/1744666X.2016.1125782

5. Federal’nye klinicheskie rekomendatsii po vedeniyu bol’nykh lokalizovannoi sklerodermiei. Russian Society of Dermatovenerologists and Cosmetologists. Moscow; 2015. (In Russ).

6. Del Galdo F, Lescoat A, Conaghan PG, et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Ann Rheum Dis. 2025;84(1):29–40. doi: https://doi.org/10.1136/ard-2024-226430

7. Suliman YA, Distler O. Novel aspects in the pathophysiology of peripheral vasculopathy in systemic sclerosis. Curr Rheumatol Rev. 2013;9(4):237–244. doi: https://doi.org/10.2174/157339710904140417123932

8. Belperio JA, Dy M, Burdick MD, et al. Interaction of IL-13 and C10 in the pathogenesis of bleomycin-induced pulmonary fibrosis. Am J Respir Cell Mol Biol. 2002;27(4):419–427. doi: https://doi.org/10.1165/rcmb.2002-0009OC

9. Rosendahl AH, Schönborn K, Krieg T. Pathophysiology of systemic sclerosis (scleroderma). Kaohsiung J Med Sci. 2022;38(3):187–195. doi: https://doi.org/10.1002/kjm2.12505

10. Proekt federal’nykh klinicheskikh rekomendatsii po vedeniyu detei s sistemnym sklerozom. Russian Society of Pediatric Rheumatologists. Moscow; 2021. (In Russ).

11. Zulian F, Martini G. Childhood systemic sclerosis. Curr Opin Rheumatol. 2007;19(6):592–597. doi: https://doi.org/10.1097/BOR.0b013e3282f01625

12. Territorial body of the Federal State Statistics Service for the Samara Region: Official website. (In Russ). Доступно по: https://63.rosstat.gov.ru. Ссылка активна на 09.04.2025.

13. Samara Oblast — what is the population in 1991–2020? In: Population of the Russian Federation: Official website. (In Russ).] Доступно по: https://численность-населения.рф/самарская-область. Ссылка активна на 09.04.2025.

14. Fernández-Codina A, Walker KM, Pope JE. Scleroderma Algorithm Group. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheumatol. 2018;70(11):1820–1828. doi: https://doi.org/10.1002/art.40560

15. Raupov RK, Imelbaev AI, Kostik MM. Juvenile Localized Scleroderma from the Perspective of Pediatric Rheumatologist. Aspects of Diagnostics. Voprosy sovremennoi pediatrii — Current Pediatrics. 2020;19(2):150–161. (In Russ). doi: https://doi.org/10.15690/vsp.v19i2.2109

16. van den Hoogen FH, Boerbooms AM, Swaak AJ, et al. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol. 1996;35(4):364–72. doi: https://doi.org/10.1093/rheumatology/35.4.364

17. Pope JE, Bellamy N, Seibold JR, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum. 2001;44(6):1351–1358. doi: https://doi.org/10.1002/1529-0131(200106)44:6<1351

18. Smith V, Herrick AL, Ingegnoli F, et al. Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud’s phenomenon and systemic sclerosis. Autoimmun Rev. 2020;19(3):102458. doi: https://doi.org/10.1016/j.aut-rev.2020.102458

19. Marrani E, Foeldvari I, Lopez JA, et al. Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach. Semin Arthritis Rheum. 2018;48(3):495–503. doi: https://doi.org/10.1016/j.semarthrit.2018.03.003

20. Trang G, Steele R, Baron M, Hudson M. Corticosteroids and the risk of scleroderma renal crisis: a systematic review. Rheumatol Int. 2012;32(3):645–653. doi: https://doi.org/10.1007/s00296-010-1697-6

21. Kumar AB, Blixt EK, Drage LA, et al. Treatment of morphea with hydroxychloroquine: A retrospective review of 84 patients at Mayo Clinic, 1996–2013. J Am Acad Dermatol. 2019;80(6):1658–1663. doi: https://doi.org/10.1016/j.jaad.2019.01.040