Anemia syndrome as the first clinical manifestation of Sjogren's disease (clinical observation)
https://doi.org/10.33667/2078-5631-2025-28-34-37
Abstract
Extracellular manifestations in Sjogren's disease are characterized by wide variability, affecting almost any organ or system, including the hematopoiesis system. Among the hematological manifestations of the disease, anemia syndrome is the most common. The article presents a clinical case of a patient in whom Sjogren's disease debuted with anemic syndrome, which led to a wide diagnostic search to clarify the diagnosis. Thus, the clinical case we observed is interesting not only from the standpoint of the importance of establishing a clinical diagnosis, but also the timeliness of treatment initiation, which affects the prognosis of the disease.
About the Authors
N. Yu. Tikhomirova
Kuban State Medical University; Regional Clinical Hospital No. 2
Russian Federation
Russian Federation
Tikhomirova Nadezhda Yu., PhD Med, associate professor at Dept of Faculty Therapy; rheumatologist at the Rheumatology Center of the Specialized Outpatient Treatment
Krasnodar
L. N. Eliseeva
Kuban State Medical University
Russian Federation
Russian Federation
Eliseeva Lyudmila N., DM Sci (habil.), professor, head of Dept of Faculty Therapy
Krasnodar
N. P. Belozerova
Kuban State Medical University; Regional Clinical Hospital No. 2
Russian Federation
Russian Federation
Belozerova Natalia P., PhD Med, associate professor at Dept of Faculty Therapy; head of the Rheumatology Center of the Specialized Outpatient Treatment
Krasnodar
E. S. Kameneva
Kuban State Medical University; Regional Clinical Hospital No. 2
Russian Federation
Russian Federation
Kameneva Elena S., PhD Med, associate professor at Dept of Polyclinic Therapy with a course of general medical practice (family medicine) Faculty of Advanced Training and Professional Retraining of Specialists; head of Outpatient Dept of Specialized Outpatient Treatment
Krasnodar
M. I. Bocharnikova
Kuban State Medical University
Russian Federation
Russian Federation
Bocharnikova Marina I., PhD Med, associate professor at Dept of Propaedeutics of Internal Diseases
Krasnodar
References
1. Qin B., Wang J., Yang Z., Yang M., Ma N., Huang F., et al. Epidemiology of Primary Sjögren's Syndrome: A Systematic Review and Meta-Analysis. Ann. Rheum. Dis. 2015; 74 (11): 1983–1989. https://doi.org/10.1136/annrheumdis-2014-205375
2. Meijer J.M., Meiners P.M., Huddleston Slater J.J., Spijkervet F.K., Kallenberg C.G., Vissink A. et al. Health-Related Quality of Life, Employment and Disability in Patients with Sjogren's Syndrome. Rheumatology (Oxford). 2009; 48 (9): 1077–1082. https://doi.org/10.1093/rheumatology/kep141
3. Parisis D., Chivasso C., Perret J., Soyfoo M.S., Delporte C. Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy. J. Clin. Med. 2020; 9: 2299. https://doi.org/10.3390/jcm9072299
4. André F., Böckle B.C. Sjögren’s syndrome. J. Dtsch. Dermatol. Ges. J. Ger. Soc. Dermatol. JDDG 2022; 20: 980–1002. https://doi.org/10.1111/ddg.14823
5. Chivasso C., Sarrand J., Perret J., Delporte C., Soyfoo M.S. The Involvement of Innate and Adaptive Immunity in the Initiation and Perpetuation of Sjögren’s Syndrome. Int. J. Mol. Sci. 2021; 22: 658. https://doi.org/10.3390/ijms22020658
6. Bombardieri M., Argyropoulou O.D., Ferro F., Coleby R., Pontarini E., Governato G., Lucchesi D., Fulvio G., Tzioufas A.G., Baldini C. One year in review 2020: Pathogenesis of primary Sjögren’s syndrome. Clin. Exp. Rheumatol. 2020; 38 (Suppl. S126): 3–9. https://doi.org/10.55563/clinexprheumatol/i8iszc
7. Greenan E,. Pilson Q., Gabhann-Dromgoole J.N., Murphy C.C. Relationship between clinical parameters and quality of life in primary Sjögren’s Syndrome: A prospective study. Eye 2023; 1: 1–8. https://doi.org/10.1038/s41433-023-02386-2
8. Seror R., Bowman S.J., Brito-Zeron P., Theander E., Bootsma H., Tzioufas A., Gottenberg J.E., Ramos-Casals M., Dörner T., Ravaud P. et al. EULAR Sjögren’s syndrome disease activity index (ESSDAI): A user guide. RMD Open 2015; 1: e000022. https://doi.org/10.1136/rmdopen-2014–000022
9. García-Carrasco M., Mendoza-Pinto C., Jiménez-Hernández C., Jiménez-Hernández M., Nava-Zavala A., Riebeling C. Serologic features of primary Sjögren’s syndrome: Clinical and prognostic correlation. Int. J. Clin. Rheumtol. 2012; 7: 651–659. https://doi.org/10.2217/ijr.12.64
10. Stergiou I.E., Kapsogeorgou E.E., Tzioufas A.G., Voulgarelis M., Goules A.V. Clinical Phenotype and Mechanisms of Leukopenia/Neutropenia in Patients with Primary Sjögren’s Syndrome. Mediterr. J. Rheumatol. 2022; 33: 99–101. https://doi.org/10.31138/mjr.33.1.99
11. Koh J.H., Lee J., Chung S.H., Kwok S.K., Park S.H. Relation of Autoimmune Cytopenia to Glandular and Systemic Manifestations in Primary Sjögren Syndrome: Analysis of 113 Korean Patients. J. Rheumatol. 2015; 42: 1817–1824. https://doi.org/10.3899/jrheum.150058
12. Mihai A., Caruntu A., Opris-Belinski D., Jurcut C., Dima A., Caruntu C., Ionescu R. The Predictive Role of Neutrophil-to-Lymphocyte Ratio (NLR), Platelet-to-Lymphocyte Ratio (PLR), Monocytes-to-Lymphocyte Ratio (MLR) and Gammaglobulins for the Development of Cutaneous Vasculitis Lesions in Primary Sjögren’s Syndrome. J. Clin. Med. 2022; 11: 5525. https://doi.org/10.3390/jcm11195525
13. Wei L., Zhifei X., Xiaoran N., Meilu L., Yang L., Yixuan L., Xiuying R., Yashuang S., Jingjing C., Shaoying G., et al. Patients with early-onset primary Sjögren’s syndrome have distinctive clinical manifestations and circulating lymphocyte profiles. Rheumatology. 2022; 61: 597–605. https://doi.org/10.1093/rheumatology/keab367
14. Malladi A.S., Sack K.E., Shiboski S.C., Shiboski C.H., Baer A.N., Banushree R., Dong Y., Helin P., Kirkham B.W., Li M., et al. Primary Sjögren’s syndrome as a systemic disease: A study of participants enrolled in an international Sjögren’s syndrome registry. Arthritis Care Res. 2012; 64: 911–918. https://doi.org/10.1002/acr.21610
15. Flores-Chávez A., Kostov B., Solans R., Fraile G., Maure B., Feijoo-Massó C., Rascón F.J., Pérez-Alvarez R., Zamora-Pasadas M., García-Pérez A. et al. Severe, life-threatening phenotype of primary Sjögren’s syndrome: Clinical characterisation and outcomes in 1580 patients (GEASSS Registry). Clin. Exp. Rheumatol. 2018; 36 (Suppl. S112): 121–129. https://doi.org/10.1136/annrheumdis-2018-eular.5114
16. Wu C., Yang P., Liu H., Xiao W., Zhao L. Increased frequency of CCR7(+)CD4(+) T cells from patients with primary Sjögren’s syndrome: An indicator of disease activity rather than of damage severity. Cytokine 2018; 110: 9–17. https://doi.org/10.1016/j.cyto.2018.04.015
17. Luo J., Song W.J., Chen J.Q., Yang G.Y., Yang J.Y., Yu X.B., Huang Z.W., Xu Y., Wang J.M., Tao Q.W. Factors associated with secondary immune thrombocytopenia in patients with primary Sjögren’s syndrome: A retrospective study of 639 cases. Clin. Exp. Rheumatol. 2022; 40: 2245–2252. https://doi.org/10.55563/clinexprheumatol/8hgmjm
18. Pugliesi A, Egypto DCSD, Duffles G, Cantali DU, Pasoto SG, Oliveira FR, Valim V, Lopes MLL, Miyamoto ST, Fernandes MLMS, Fialho SCMS, Pinheiro AC, Santos LCD, Appenzeller S, Ribeiro SLE, Libório-Kimura TN, Santos MCLFS, Gennari JDA, Pernanbuco R, Capobiano KG, Civile VT, Pinto ACPN, Rocha-Filho CR, Rocha APD, Trevisani VFM. Recommendations on cutaneous and hematological manifestations of Sjögren's disease by the Brazilian Society of Rheumatology. Adv Rheumatol. 2024; 64 (1): 51. https://doi.org/10.1186/s42358-024-00391-x
19. Zhou JG, Qing YF, Jiang L, Yang QB, Luo WF. Clinical analysis of primary Sjögren’s syndrome complicating anemia. Clin Rheumatol. 2010; 29 (5): 525–29. https://doi.org/10.1007/s10067-009-1366-x
20. Ter Borg E.J., Kelder J.C. Is extra-glandular organ damage in primary Sjögren’s syndrome related to the presence of systemic auto-antibodies and/or hypergammaglobulinemia? A long-term cohort study with 110 patients from the Netherlands. Int. J. Rheum. Dis. 2017; 20: 875–88. https://doi.org/10.55563/clinexprheumatol/8hgmjm
21. Goules A.V., Tzioufas A.G. Lymphomagenesis in Sjögren’s syndrome: Predictive biomarkers towards precision medicine. Autoimmun. Rev. 2019; 18: 137–143. https://doi.org/10.1016/j.autrev.2018.08.007
22. Sebastian A., Madej M., Sebastian M., Butrym A., Woytala P., Hałoń A., Wiland P. Prevalence and clinical presentation of lymphoproliferative disorder in patients with primary Sjögren’s syndrome. Rheumatol. Int. 2020; 40: 399–404. https://doi.org/10.1007/s00296-020-04522-7
23. Ramos-Casals M., Brito-Zerón, P., Seror, R., Bootsma, H., Bowman, S.J., Dörner, T., Gottenberg J.E., Mariette X., Theander E., Bombardieri S. et al. Characterization of systemic disease in primary Sjögren’s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology. 2015; 54: 2230–2238. https://doi.org/10.1093/rheumatology/kev200
24. Retamozo S., Brito-Zerón P., Ramos-Casals M. Prognostic markers of lymphoma development in primary Sjögren syndrome. Lupus 2019; 28: 923–936. https://doi.org/10.1177/0961203319857132
25. Bowman S. J. Primary Sjögren’s syndrome. Lupus 2018; 27: 32–35. https://doi.org/10.1177/0961203318801673
26. Manfrè V., Giovannini I., ZandonellaCallegher S., Lorenzon M., Pegolo E., Tel A., Gandolfo S., Quartuccio L., De Vita S., Zabotti A. Ultrasound and Bioptic Investigation of Patients with Primary Sjögren’s Syndrome. J. Clin. Med. 2021; 10: 1171. https://doi.org/10.3390/jcm10061171
27. Baldini C., Zabotti A., Filipovic N., Vukicevic A., Luciano N., Ferro F., Lorenzon M., De Vita S. Imaging in primary Sjögren’s syndrome: The ‘obsolete and the new’. Clin. Exp. Rheumatol. 2018; 36 (Suppl. S112): 215–221. https://doi.org/10.1007/s10067-016-3499-z
28. Nocturne G., Mariette X. B cells in the pathogenesis of primary Sjögren syndrome. Nat. Rev. Rheumatol. 2018; 14: 133–145. https://doi.org/10.1038/nrrheum.2018.1
29. Nocturne, G., Pontarini, E., Bombardieri, M., Mariette, X. Lymphomas complicating primary Sjögren’s syndrome: From autoimmunity to lymphoma. Rheumatology 2021; 60: 3513–3521. https://doi.org/10.1093/rheumatology/kez052
30. Bautista-Vargas M., Vivas A.J., Tobón G.J. Minor salivary gland biopsy: Its role in the classification and prognosis of Sjögren’s syndrome. Autoimmun. Rev. 2020; 19: 102690.https://doi.org/10.1016/j.autrev.2020.102690
31. Retamozo S., Sisó-Almirall A., Flores-Chávez A., Ramos-Casals M., Brito-Zerón P. An update of targeted therapeutic options for primary Sjögren syndrome: Current status and future development. Expert Opin. Pharm. 2021; 22: 2359–2371. https://doi.org/10.1080/14656566.2021.1951224
32. Priori R., Mastromanno L., Izzo R. What about glucocorticoids in primary Sjögren’s syndrome? Clin. Exp. Rheumatol. 2020; 38 (Suppl. S126): 237–244. https://doi.org/10.1007/s00296-021-04899-z
33. Suarez-Almazor M.E., Belseck E., Shea B., Homik J., Wells G., Tugwell P. Antimalarials for Treating Rheumatoid Arthritis. Cochrane Database Syst. Rev. 2000; 4. https://doi.org/10.1002/14651858.cd000959
34. Ruiz-Irastorza G., Ramos-Casals M., Brito-Zeron P., Khamashta M.A. Clinical Efficacy and Side Effects of Antimalarials in Systemic Lupus Erythematosus: A Systematic Review. Ann. Rheum. Dis. 2010; 69 (1): 20–28. https://doi.org/10.1136/ard.2008.101766
35. Schrezenmeier E., Dörner T. Mechanisms of Action of Hydroxychloroquine and Chloroquine: Implications for Rheumatology. Nat. Rev. Rheumatol. 2020; 16 (3): 155–166. https://doi.org/10.1038/s41584-020-0372-x
36. Fox R.I., Dixon R., Guarrasi V., Krubel S. (1996). Treatment of Primary Sjögren's Syndrome with Hydroxychloroquine: A Retrospective, Open-Label Study. Lupus 1996; 5 (Suppl. 1): S31–S36. https://doi.org/10.1177/0961203396005001081
37. Tishler M., Yaron I., Shirazi I., Yaron M. (1999). Hydroxychloroquine Treatment for Primary Sjögren's Syndrome: Its Effect on Salivary and Serum Inflammatory Markers. Ann. Rheum. Dis. 1999; 58 (4): 253–256. https://doi.org/10.1136/ard.58.4.253
38. Rihl M., Ulbricht K., Schmidt R.E., Witte T. Treatment of Sicca Symptoms with Hydroxychloroquine in Patients with Sjogren'sSyndrome. Rheumatology (Oxford). 2009; 48 (7): 796–799. https://doi.org/10.1093/rheumatology/kep104
39. Çankaya H., Alpöz E., Karabulut G., Güneri P., Boyacioglu H., Kabasakal Y. Effects of Hydroxychloroquine on Salivary Flow Rates and Oral Complaints of Sjögren Patients: A Prospective Sample Study. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 2010; 110 (1): 62–67. https://doi.org/10.1016/j.tripleo.2010.02.032
40. Demarchi J., Papasidero S., Medina M.A., Klajn D., Chaparro Del Moral R., Rillo O. et al. (2017). Primary Sjögren's Syndrome: ExtraglandularManifestations and Hydroxychloroquine Therapy. Clin. Rheumatol. 2017; 36 (11): 2455–2460. https://doi.org/10.1007/s10067-017-3822-3
41. Hernández-Molina G., Valim V., Secco A., Atisha-Fregoso Y., Guerra E., Adrover M. et al. Do antimalarialsProtect against Damage Accrual in Primary Sjögren's Syndrome? Results from a Latin-American Retrospective Cohort. Clin. Exp. Rheumatol. 2018; 36 (Suppl. 112): 182–185. https://doi.org/10.1093/geroni/igy023.1873
42. Xu D., Zhao S., Li Q., Wang Y.H., Zhao J. L., Li M. T. et al. (2020). Characteristics of Chinese Patients with Primary Sjögren's Syndrome: Preliminary Report of a Multi-Centre Registration Study. Lupus2020; 29 (1): 45–51. https://doi.org/10.1177/0961203319889666
Review
For citations:
Tikhomirova N.Yu., Eliseeva L.N., Belozerova N.P., Kameneva E.S., Bocharnikova M.I. Anemia syndrome as the first clinical manifestation of Sjogren's disease (clinical observation). Medical alphabet. 2025;(28):34-37. (In Russ.) https://doi.org/10.33667/2078-5631-2025-28-34-37
Views:
210
JATS XML
Email this article 