Vol 10, No 1 (2024)
Case report
Published online: 2024-01-24

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Primary Sjögren’s syndrome complicated by development of B-cell lymphoma

Magdalena Romanowska1, Magdalena Krajewska-Włodarczyk2
Rheumatology Forum 2024;10(1):38-42.


Sjögren’s syndrome is a chronic autoimmune inflammatory connective tissue disease of unknown aetiology. It is marked by lymphocytic infiltration of exocrine glands (mainly lacrimal glands and salivary glands), which impairs their function and leads, among other things, to symptoms of dryness that are typical of this syndrome. In addition to glandular lesions, other organs and systems such as the gastrointestinal tract, nervous system and vasculitis may also be involved. There is also a significantly higher incidence of lymphomas in primary Sjögren’s syndrome compared to the general population. This paper presents a case report of a 63-year-old female patient with primary Sjögren’s syndrome who presented with a worsening of her condition and the development of B-cell lymphoma in the course of her disease.

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  1. Maślińska M, Przygodzka M, Kwiatkowska B, et al. Sjögren's syndrome: still not fully understood disease. Rheumatol Int. 2015; 35(2): 233–241.
  2. Bombardieri M, Argyropoulou OD, Ferro F, et al. One year in review 2020: pathogenesis of primary Sjögren's syndrome. Clin Exp Rheumatol. 2020; 38 Suppl 126(4): 3–9.
  3. Nocturne G, Virone A, Ng WF, et al. Rheumatoid Factor and Disease Activity Are Independent Predictors of Lymphoma in Primary Sjögren's Syndrome. Arthritis Rheumatol. 2016; 68(4): 977–985.
  4. Shimizu T, Nakamura H, Kawakami A. Role of the Innate Immunity Signaling Pathway in the Pathogenesis of Sjögren's Syndrome. Int J Mol Sci. 2021; 22(6).
  5. Delaleu N, Jonsson R, Koller MM. Sjögren's syndrome. Eur J Oral Sci. 2005; 113(2): 101–113.
  6. Madaliński K, Dzierzanowska-Fangrat K, Jóźwiak P, et al. Rola zakazeń wirusem zapalenia wątroby typu C w patogenezie zespołu Sjögrena. Pol Arch Med Wewn. 2002; 107(2): 167–171.
  7. Lendrem D, Mitchell S, McMeekin P, et al. UK primary Sjögren's Syndrome Registry. Health-related utility values of patients with primary Sjögren's syndrome and its predictors. Ann Rheum Dis. 2014; 73(7): 1362–1368.
  8. Skopouli FN, Dafni U, Ioannidis JP, et al. Clinical evolution, and morbidity and mortality of primary Sjögren's syndrome. Semin Arthritis Rheum. 2000; 29(5): 296–304.
  9. Chivasso C, Sarrand J, Perret J, et al. The Involvement of Innate and Adaptive Immunity in the Initiation and Perpetuation of Sjögren's Syndrome. Int J Mol Sci. 2021; 22(2).
  10. Theander E, Henriksson G, Ljungberg O, et al. Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006; 65(6): 796–803.
  11. Gottenberg JE, Lavie F, Abbed K, et al. CD4 CD25high regulatory T cells are not impaired in patients with primary Sjögren's syndrome. J Autoimmun. 2005; 24(3): 235–242.
  12. Fox RI, Luppi M, Kang HI, et al. Detection of Epstein-Barr virus-associated antigens and DNA in salivary gland biopsies from patients with Sjogren's syndrome. J Immunol. 1986; 137(10): 3162–3168.
  13. Wen S, Shimizu N, Yoshiyama H, et al. Association of Epstein-Barr virus (EBV) with Sjögren's syndrome: differential EBV expression between epithelial cells and lymphocytes in salivary glands. Am J Pathol. 1996; 149(5): 1511–1517.
  14. Ramos-Casals M, Brito-Zerón P, Font J. The overlap of Sjögren's syndrome with other systemic autoimmune diseases. Semin Arthritis Rheum. 2007; 36(4): 246–255.
  15. Rasmussen A, Ice JA, Li He, et al. Comparison of the American-European Consensus Group Sjogren's syndrome classification criteria to newly proposed American College of Rheumatology criteria in a large, carefully characterised sicca cohort. Ann Rheum Dis. 2014; 73(1): 31–38.
  16. Shiboski CH, Shiboski SC, Seror R, et al. International Sjögren's Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017; 69(1): 35–45.
  17. Nocturne G, Mariette X. Sjögren Syndrome-associated lymphomas: an update on pathogenesis and management. Br J Haematol. 2015; 168(3): 317–327.
  18. Alunno A, Leone MC, Giacomelli R, et al. Lymphoma and Lymphomagenesis in Primary Sjögren's Syndrome. Front Med (Lausanne). 2018; 5: 102.
  19. Świerkocka K, Łącki JK. Chłoniaki w zespole Sjögrena. Reumatologia. 2008; 46: 16–20.
  20. Theander E, Vasaitis L, Baecklund E, et al. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren's syndrome. Ann Rheum Dis. 2011; 70(8): 1363–1368.
  21. Ioannidis JPA, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome. Arthritis Rheum. 2002; 46(3): 741–747.
  22. Gasparotto D, De Vita S, De Re V, et al. Extrasalivary lymphoma development in Sjögren's syndrome: clonal evolution from parotid gland lymphoproliferation and role of local triggering. Arthritis Rheum. 2003; 48(11): 3181–3186.
  23. Manganelli P, Fietta P, Quaini F. Hematologic manifestations of primary Sjögren's syndrome. Clin Exp Rheumatol. 2006; 24(4): 438–448.