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КОМПЛЕКСНЫЙ ПОДХОД К ДИАГНОСТИКЕ И ЛЕЧЕНИЮ БОЛЕЗНИ ШЕГРЕНА
Агамурадов С.Р., Касумова Ф.Н., Гудратли Х.Б.


DOI: 10.61775/2413-3302.v2i40.15


РЕЗЮМЕ
Болезнь Шегрена — хроническое системное аутоиммунное заболевание, характеризующееся поражением экзокринных желёз и развитием симптомов сухости слизистых оболочек, а также возможными системными проявлениями. Диагностика нередко затруднена из-за неспецифичности симптомов и вариабельности течения. В статье представлен клинический случай пациентки с болезнью Шегрена, демонстрирующий типичные диагностические сложности и необходимость междисциплинарного подхода. Подчёркивается значение комплексной оценки клинических, лабораторных и инструментальных данных для своевременной постановки диагноза и выбора оптимальной терапии.
Keywords: болезнь Шегрена, сухой синдром, ксерофтальмия, ксеростомия, анти-Ro/SSA и анти-La/SSB антитела


ЛИТЕРАТУРА
  1. Hochberg M.C., Gravallese E.M., Smolen J.S., van der Heijde D., Weinblatt M.E., Weisman M.H. Rheumatology, 2-Volume Set, 8th Edition, Elsevier Editors: 2023, 2034 p.
  2. Negrini S, Emmi G, Greco M, Borro M, Sardanelli F, Murdaca G, Indiveri F, Puppo F. Sjögren's syndrome: a systemic autoimmune disease. Clin Exp Med. 2022; 22(1):9-25. doi: 10.1007/s10238-021-00728-6
  3. Firestein GS, Budd RC, Gabriel SE, McInnes IB, O’Dell JR, eds. Firestein & Kelley’s Textbook of Rheumatology. 12th ed. Elsevier; 2025; 2876 p.
  4. Ji J, Sundquist J, Sundquist K. Gender-specific incidence of autoimmune diseases fromnational registers. J Autoimmun 2016; 69: 102–106. doi: 10.1016/j.jaut.2016.03.003
  5. Yazisiz V, Gocer M, Erbasan F et al. Survival analysis of patients with Sjogren’s syn-drome in Turkey: a tertiary hospital-based study. Clin Rheumatol 2020; 39(1): 233–241. doi: 10.1007/s10067-019-04744-6
  6. Weng MY, Huang YT, Liu MF, Lu TH. Incidence and mortality of treated primary Sjo-gren’s syndrome in Taiwan: a population-based study. J Rheumatol 2011; 38(4): 706–708. doi: 10.3899/jrheum.100883
  7. Verstappen GM, Pringle S, Bootsma H, Kroese FGM. Epithelial-immune cell interplayin primary Sjogren syndrome salivary gland pathogenesis. Nat Rev Rheumatol 2021;17(6): 333–348. doi: 10.1038/s41584-021-00605-2
  8. Bombardieri M, Argyropoulou OD, Ferro F et al. One year in review 2020: pathogenesis of primary Sjogren’s syndrome. Clin Exp Rheumatol. 2020; 38 (Suppl 126(4)): 3–9.
  9. Ramos-Casals M., Brito-Zerón P., Sisó-Almirall A., Bosch X. Primary Sjogren syndrome. BMJ, 2012; 14;344: e3821. doi: 10.1136/bmj.e3821
  10. Mavragani CP, Crow MK. Activation of the type I interferon pathway in primary Sjogren's syndrome. J Autoimmun. 2010; 35(3):225-31. doi: 10.1016/j.jaut. 2010. 06.012.
  11. André F, Böckle BC. Sjögren's syndrome. J Dtsch Dermatol Ges. 2022 Jul;20(7):980-1002. doi: 10.1111/ddg.14823
  12. Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M. Sjögren syndrome. Nat Rev Dis Primers. 2016; 2:16047. doi: 10.1038/nrdp.2016.47
  13. Baldini C, Pepe P, Quartuccio L, et al. Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology. 2014;53(5):839–844. doi: 10.1093/rheumatology/ket427
  14. Brito-Zeron P, Theander E, Baldini C, et al. Early diagnosis of primary Sjogren's syndrome: EULAR-SS task force clinical recommendations // Exp Rev Clin Immunol. 2016;12(2):137–156. doi: 10.1586/1744666X.2016.1109449
  15. Kuklinski E, Asbell PA. Sjogren's syndrome from the perspective of ophthalmology. Clin Immunol. 2017; 182:55–61. doi: 10.1016/j.clim.2017.04.017
  16. Fauchais AL, Ouattara B, Gondran G et al. Articular manifestations in primary Sjogren’s syndrome: clinical significance and prognosis of 188 patients. Rheumatology (Oxford) 2010; 49(6): 1164–72. doi: 10.1093/rheumatology/keq047
  17. Ramos-Casals M, Brito-Zeron P, Seror R, et al. Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements // Rheumatology. 2015;54(12):2230–2238. doi: 10.1093/rheumatology/kev200
  18. Cafaro, G., Baldini, C., Bombardieri, S., Carubbi, F. Peripheral nervous system involvement in Sjögren’s syndrome: Analysis of a cohort from the Italian Research Group on Sjögren’s Syndrome. Frontiers in Immunology, 2021;12, 615656. doi: 10.3389/fimmu.2021.615656
  19. Liampas A., Parperis K., Erotocritou M.F. et al. Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis // Eur J Neurol. 2023; 30(1):255-265. doi: 10.1111/ene.15555
  20. Pavlakis P.P., Alexopoulos H., Kosmidis M.L. et al. Peripheral neuropathies in Sjögren's syndrome: a critical update on clinical features and pathogenetic mechanisms // J Autoimmun., 2012;39(1-2):27-33. doi: 10.1016/j.jaut.2012.01.003.
  21. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American college of rheumatology/European league against rheumatism classification criteria for primary Sjogren's syndrome: a consensus and data-driven methodology involving three international patient cohorts // Arthritis Rheumatol. 2017;69(1):35–45. doi: 10.1002/art.39859
  22. Whitcher JP, Shiboski CH, Shiboski SC, et al. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjogren's Syndrome International Registry. Am J Ophthalmol. 2010;149(3):405–415. doi: 10.1016/j.ajo.2009.09.013.
  23. Patel R, Shahane A. The epidemiology of Sjogren's syndrome // Clin Epidemiol. 2014; 6:247–255. doi: 10.2147/CLEP.S47399
  24. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American college of rheumatology/European league against rheumatism classification criteria for primary Sjogren's syndrome: a consensus and data-driven methodology involving three international patient cohorts // Arthritis Rheumatol. 2017;69(1):35–45. doi: 10.1002/art.39859
  25. Ramos-Casals M, Brito-Zeron P, Bombardieri S, et al. EULAR recommendations for the management of Sjogren's syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3–18. doi: 10.1136/annrheumdis-2019-216114