XÜLASƏ
Şjögren (Birincili Sjögren sindromu) xəstəliyi — ekzokrin vəzilərin zədələnməsi, selikli qişaların quruluğu və müxtəlif sistemli təzahürlərlə xarakterizə olunan xroniki sistemli autoimmun xəstəlikdir. Simptomların qeyri-spesifikliyi və xəstəliyin dəyişkən gedişi diaqnostikanı çətinləşdirir. Şjöqren xəstəliyindən əziyyət çəkən pasiyentin klinik halı təqdim olunur ki, bu da diaqnostik çətinlikləri və multidissiplinar yanaşmanın əhəmiyyətini əks etdirir. Klinik, laborator və instrumental göstəricilərinin kompleks qiymətləndirilməsinin vaxtında diaqnoz və optimal müalicə seçimi üçün vacibliyi vurğulanır.
Açar sözlər: Şjögren sindromu, quru sindrom, kseroftalmiya; kserostomiya, anti-Ro/SSA və anti-La/SSB anticismləri

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ƏDƏBİYYAT

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