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Sjögren’s syndrome

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Abstract

Sjögren’s syndrome (SS) is characterised as keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth) commonly associated with salivary gland enlargement, and is referred to as Primary Sjögren’s syndrome. It is known as Secondary Sjögren’s syndrome when it occurs in patients, with connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, polyarthritis nodosa, polymyositis, and systemic sclerosis. SS has also been associated with chronic graft-versus-host disease after allogeneic bone marrow transplantation, human immunodeficiency syndrome (AIDS), hepatitis C infection (HCV), chronic biliary cirrhosis, neoplastic and myeloplastic syndromes, fibromyalgia, and chronic fatigue syndrome.

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References

  • Agman-Levin N, Kivity S, Tzioufas AG et al (2012) Low levels vitamin D are associated with neuropathy and lymphoma among patients with Sjögren’s syndrome. J Autoimmun 39:234–239

    Article  Google Scholar 

  • Albrich K (1928) Filiform keratitis due to insufficient secretion of lacrimal glands. 3 cases. Arch Ophthal 121:402–410

    Google Scholar 

  • Ambrosi A, Salomonsson S, Eliasson H et al (2012) Development of heart block in children of SSA/SSB—autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern. Ann Rheum Dis 71:334–340

    Article  PubMed  Google Scholar 

  • Baldini C, Luciano N, Tarantini G, Pascale R, Sernissi F, Mosca M, Caramella D, Bombardieri S (2015) Salivary gland ultrasonography: a highly specific tool for the early diagnosis of primary Sjögren’s syndrome. Arthritis Res Ther 17(1):146–154

    Article  PubMed  PubMed Central  Google Scholar 

  • Beck JS, Anderson JR, Bloch KJ et al (1965) Antinuclear and precipitating autoantibodies in Sjögren’s syndrome. Ann Rheum Dis 24:16–22

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Betsch A (1928) Die chronische Keratitis filiformis als Folge mangelnder Tränensekretion. Klin Mbl Augenheilk 80:618–623

    Google Scholar 

  • Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ (1965) Sjögren’s syndrome. A clinical, pathological, and serological study of sixty-two cases. Medicine (baltimore) 44:187–231

    Article  CAS  PubMed  Google Scholar 

  • Bowman SJ, Booth DA, Platts RG et al (2003) Validation of the sicca symptoms inventory for clinical studies of Sjögren’s syndrome. J Rheumatol 30(6):1259–1266

    PubMed  Google Scholar 

  • Bowman SJ, Booth DA, Platts RG (2004) Measurement of fatigue and discomfort in primary Sjögren’s syndrome using a new questionnaire tool. Rheumatology (oxford) 43(6):758–764

    Article  CAS  PubMed  Google Scholar 

  • Buchanan WW (1997) Sjögren’s syndrome: the persisting conundrum. J Rheumatol 24:1667–1669

    CAS  PubMed  Google Scholar 

  • Cantaert T, Baeten D, Tak PT, van Baarsen LGM (2010) Type I IFN and TNFα cross-regulation in immune-mediated inflammatory disease: basic concepts and clinical relevance. Arthritis Res Ther 12:219

    Article  PubMed  PubMed Central  Google Scholar 

  • Chisholm DM, Mason DK (1968) Labial salivary gland biopsy in Sjögren’s syndrome. J Clin Pathol 21:656–660

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ciccia F, Accardo-Palumbo A, Alessandro R, Alessandri C, Priori R, Guggino G, Raimondo S, Carubbi F, Valesini G, Giacomelli R, Rizzo A, Triolo G (2015) Interleukin-36α axis is modulated in patients with primary Sjögren’s syndrome. Clin Exp Immunol 181(2):230–238

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Daniels TE, Aufdemorte TB, Greenspan JS (1987) Histopathology of Sjögren’s syndrome. In: Talal N, Moutsopoulos HM, Kassan SS (eds) Sjögren’s syndrome: clinical and immunological aspects. Springer-Verlag, Berlin, pp 266–286

  • Fauchais Al, Martel C, Gondran G et al (2010) Immunological profile in primary Sjögren syndrome: clinical significance, prognosis and long-term evolution to other auto-immune disease. Autoimmun Rev 9:595–599

    Article  CAS  PubMed  Google Scholar 

  • Ferro F, Vagelli R, Bruni C et al (2016) One year in review 2016: Sjögren’s syndrome. Clin Exp Rheumatol 34:161–171

    PubMed  Google Scholar 

  • Fuchs A (1919) Ein Fall von Fehlen der Tränen- und Mundspeichel-Sekretion. A Augenh 42:253–254

    Google Scholar 

  • García-Carrasco M, Ramos M, Cervera R et al (1997) Hepatitis C virus infection in ‘primary’ Sjögren’s syndrome: prevalence and clinical significance in a series of 90 patients. Ann Rheum Dis 56:173–175

    Article  PubMed  PubMed Central  Google Scholar 

  • Gougerot H (1926) Insuffisance progressive et atrophie dess glandes salivaires et muqueuses de la bouche, des conjenctives (et parfois des muqueuses nasale, laryngee, vulvainre) secheresse de la bouche, des conjunctives, et. Bull Med (paris) 40:360–368

    Google Scholar 

  • Hadden WB (1888) On “dry mouth”, or suppression of the salivary and buccal secretions. Trans Clin Soc London 21:176–179

    Google Scholar 

  • Hickish T, Cunningham D, Colston K et al (1993) The effect of 1,25-dihydroxyvitamin D3 on lymphoma cell lines and expression of vitamin D receptor in lymphoma. Br Cancer 68:668–672

    Article  CAS  Google Scholar 

  • Houwer AWM (1927) Keratitis fermentosa and chronic arthritis. Trans Ophthal Soc, UK 47:88–96

    Google Scholar 

  • Isakowitz J (1928) Die Endokrine Periarthritis (Uber) und Keratitis filiformis. Klin Monatsblätter Augenheilkunde 81:85–86

    Google Scholar 

  • Kay RA, Hay EM, Dyer PA et al (1991) An abnormal T cell repertoire in hypergammaglobulinaemic primary Sjögren’s syndrome. Clin Exp Immunol 85:262–264

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kincaid MC (1987) The eye in Sjögren’s syndrome. In: Talal N, Moutsopoulos HM, Kassam SS (eds) Sjögren’s syndrome: clinical and Immunological Aspects. Springer-Verlag, Berlin, pp 25–34

    Chapter  Google Scholar 

  • Leber T (1982) Ueber die Entstehung der Netzhautablösung. Ber Versamml Ophthal Ges Stuttg 14:164–166

    Google Scholar 

  • Luppi F, Sebastiani M, Silva M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A (2020) Interstitial lung disease in Sjögren’s syndrome: a clinical review. Clin Exp Rheumatol 38(Suppl 126):291–300

    PubMed  Google Scholar 

  • Mariette X, Seror R, Quartuccio L, Baron G, Salvin S, Fabris M, Desmoulins F, Nocturne G, Ravaud P, De Vita S (2015) Efficacy and safety of belimumab in primary Sjögren’s syndrome: results of the BELISS open-label phase II study. Ann Rheum Dis 74(3):526–531

    Article  CAS  PubMed  Google Scholar 

  • Martin T, Weber JC, Levalloi H et al (2000) Salivary gland lymphomas in patients with Sjögren’s syndrome may frequently develop from rheumatoid factor B cells. Arthritis Rheum 43:908–916

    Article  CAS  PubMed  Google Scholar 

  • Maslinska M, Przygodzka M, Kwiatkowska B, Sikorska-Siudek K (2015) Sjögren’s syndrome: still not fully understood disease. Rheumatol Int 35:233–241

    Article  CAS  PubMed  Google Scholar 

  • Morgan WS, Castleman B (1953) A clinicopathologic study of “Mikulicz’s disease.” Amer J Path 29:471–503

    CAS  PubMed  PubMed Central  Google Scholar 

  • Moutsopoulos HM (1994) Sjögren’s syndrome: autoimmune epithelitis. Clin Immunol Immunopath 72:162–165

    Article  CAS  Google Scholar 

  • Moutsopoulos HM, Webber BL, Vlagopoulos TP et al (1979) Differences in the clinical manifestations of sicca syndrome in the presence and absence of rheumatoid arthritis. Am J Med 66:733–736

    Article  CAS  PubMed  Google Scholar 

  • Nezos A, Gravani F, Tassidou A, Kapsogeorgou EK, Voulgarelis M, Koutsilieris M, Crow MK, Mavragani CP (2015) Type I and II interferon signatures in Sjogren’s syndrome pathogenesis: contributions in distinct clinical phenotypes and Sjogren’s related lymphomagenesis. J Autoimmun 63:47–58

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Osler W (1892) Principles and practice of medicine. D. Appleton and Co., New York, p 328. (Reprinted by the Classics of Medicine Library, Division of Gryphon Editions, Birmingham, Alabama USA, 1978)

  • Park AL, Buchanan WW (1998) Sjögren”s syndrome: history, clinical and pathological features. Immunopharmacology 6:271–287

    Google Scholar 

  • Park WM, Mason DK (1966) Hydrostatic sialography. Radiology 86:116–122

    Article  CAS  PubMed  Google Scholar 

  • Parrago G, Rain GD, Bronchierion C, Rocher F (1987) Scintigraphy of the salivary glands in Sjögren’s syndrome. J Clin Pathol 40:1463–1467

    Article  Google Scholar 

  • Pflugfelder SC, Tseng SC, Yoshino K et al (1997) Correlation of goblet cell density and mucosal epithelial membrane mucin expression with rose Bengal staining in patients with ocular irritation. Opthalmol 104:223–235

    Article  CAS  Google Scholar 

  • Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, Granata G, Ng W-F, Fisher BA, Bombardieri M, Astorri E, Empson B, Larkin G, Crampton B, Bowman SJ, on behalf of the British Society for Rheumatology Standards, Guideline and Audit Working Group (2017) The British Society for Rheumatology guideline for the management of adults with primary Sjögren’s Syndrome. Rheumatology 56(10):e24–e48

    Article  CAS  PubMed  Google Scholar 

  • Quartuccio L, Isola M, Baldini C et al (2015) Clinical and biological differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren’s syndrome: results of a large multicentre study. Scand J Rheumatol 44:36–41

    Article  CAS  PubMed  Google Scholar 

  • Ramon-Casals M, Brito-Zeron P, Kostov B et al (2015) Google-driven search for big data in autoimmune geoepidemiology: analysis of 394,827 patients with systemic autoimmune diseases. Autoimmun Rev 14:670–679

    Article  Google Scholar 

  • Reveille JD, MacLeod MJ, Whittington K, Arnett FC (1991) Specific amino acid residues in the second hypervariable region of HLA-DQA1 and DAB1 chain genes promote the Ro(SSA)/La(SSB) autoantibody responses. J Immunol 146:3871–3875

    Article  CAS  PubMed  Google Scholar 

  • Salliot C, Gottenberg JE, Bengoufa D et al (2007) Anticentromere antibodies identify patients with Sjögren’s syndrome and autoimmune overlap syndrome. J Rheumatol 34:2253–2258

    CAS  PubMed  Google Scholar 

  • Sankar V, Brennan MT, Kok MR et al (2004) Etanercept in Sjögren’s syndrome: a twelve-week randomized, double-blind, placebo-controlled pilot clinical trial. Arthritis & Rheumatology 50(7):2240–2245

    Article  CAS  Google Scholar 

  • Schaffer AJ, Jacobsen AW (1927) Mikulicz’s syndrome: a report of ten cases. Am J Dis Child 34:327–346

    Article  Google Scholar 

  • Schirmer OWA (1903) Studien zur Physiologie und Pathologie der Tränenabfuhr. Albrecht Von Graefess Archiv Kil Exp Ophthalmol 56:197–291

    Article  Google Scholar 

  • Schöninger L (1924) Ueber Keratitis filiformis bei Hypofunktion der Tränendrüsen. Klin Mbl Augenheilk 73:208–210

    Google Scholar 

  • Schur PH (1995) Review: genetics of systemic lupus erythematosus. Lupus 4:425–437

    Article  CAS  PubMed  Google Scholar 

  • Scofield RH, Fayyaz A, Kurien B, Koelsch KA (2018) Prognostic value of Sjögren’s syndrome autoantibodies. J Lab Prec Med 3:92–103

    Article  Google Scholar 

  • Seror L, Ravaud P, Bowman SJ et al (2010a) Validation of EULAR Sjögren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren’s syndrome. Ann Rheum Dis 69(6):1103–1109

    Article  PubMed  Google Scholar 

  • Seror R, Mariette X, Bowman S et al (2010b) Accurate detection of changes in disease activity in primary Sjögren’s syndrome by the European league against rheumatism Sjögren’s syndrome disease activity index. Arthritis Care & Research (hoboken) 62(4):551–558

    Article  Google Scholar 

  • Seror R, Theander E, Brun JG et al (2013) Validation of EULAR primary Sjögren’s syndrome disease activity and patient indexes. Arthritis Rheum 64(10):S1078

    Google Scholar 

  • Shiboski CH, Shiboski SC, Seror R et al (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 Rheum. 2017 Jan;69(1):35–45 https://doi.org/10.1002/art.39859. Epub 2016 Oct 26. PMID: 27785888; PMCID: PMC5650478

    Article  PubMed  Google Scholar 

  • Sjögren H (1933) Zur Kenntnis der Keratoconjunctivitis sicca (Keratitis filiformis bei Hypofunktion der Tränendrüsen) Acta Ophthal (Klh) 11:1–151. (Translated by J.B. Hamilton, Tasmania, Australia. A New Conception of Keratoconjunctivitis Sicca (Keratitis Filiformis in Hypofunction of the Lacrimal Glands). Sydney Australia. Australian Medical Publishing Company, 1943)

  • Skopouli FN, Siouna-Fatourou NI, Ziciadis C, Moutsopoulos HM (1989) Evaluation of unstimulated whole saliva flow rate and stimulated parotid flow as confirmatory tests for xerostomia. Clin Exper Rheumatol 7:127–129

    CAS  Google Scholar 

  • Strand V, Talal N (1980) Advances in the diagnosis and concept of Sjögren’s syndrome (autoimmune exocrinopathy). Bull Rheum Dis 30:1046–1052

    CAS  Google Scholar 

  • Talal N (ed) (1989). Academic Press, London

    Google Scholar 

  • Talal N (1994) Historical overview of Sjögren’s syndrome. Clin Exper Rheumatol 12(Suppl 11):3–4

    Google Scholar 

  • Talal N, Bunim JJ (1964) The development of malignant lymphoma in the course of Sjögren’s syndrome. Amer J Med 36:529–540

    Article  CAS  PubMed  Google Scholar 

  • Talal N, Moutsopoulos HM, Kassam SS (eds) (1987). Springer-Verlag, Heidelberg

    Google Scholar 

  • Tincani A, Andreoli L, Cavazzana I et al (2013) Novel aspects of Sjögren’s syndrome in 2012. BMC Med 11:93

    Article  PubMed  PubMed Central  Google Scholar 

  • Tung CH, Chen YC, Chen YC (2022) Association between anti-hepatitis C viral intervention therapy and risk of Sjögren’s syndrome: a national retrospective analysis. J Clin Med 11(15):4259–4270

    Article  PubMed  PubMed Central  Google Scholar 

  • Tzioufas AG, Moutsopoulos HM, Talal N (1987) Lymphoid malignancy and monoclonal proteins. In: Talal N, Mautsopoulos HM, Kassam SS (eds) Sjögren’s syndrome: clinical and immunological aspects. Springer-Verlag, Berlin, pp 129–136

    Chapter  Google Scholar 

  • Vitali C, Bombardieri S, Moutsopoulos HM et al (1993) Preliminary criteria for the classification of Sjögren’s syndrome: results of a prospective concerted action supported by the European Community. Arthritis Rheum 36:340–347

    Article  CAS  PubMed  Google Scholar 

  • Vitali C, Moutsopoulos HM, Bombardieri S et al (1994) The European Community study Group on diagnostic criteria for Sjögren‘s syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjögren’s syndrome. Ann Rheum Dis 53:637–647

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • von Mikulicz-Radecki J (1892) Ueber eine eigenartige symmetrische Erkrankung des Tränen – und Mundspeicheldrüsen. In: Beitrage zur Chirurgie. Festschrift Gewid. Stuttgart. T Bilroth, pp 610–630. (English translation by J.B. Hamilton: Concerning a peculiar symmetrical disease of the lacrimal and salivary glands. Med Classics 2:165–186 1937)

  • Whaley K, Chisholm DM, Downie WW et al (1968) Lymphocytic sialedenitis in the buccal mucosa in Sjögren’s disease, rheumatoid arthritis and other arthritides; a clinical and laboratory study. Acta Rheum Scand 14:298–308

    Article  CAS  PubMed  Google Scholar 

  • Whaley K, Blair S, Low PS et al (1972) Sialographic abnormalities in Sjögren’s syndrome, rheumatoid arthritis and other arthritides and connective tissue disease: a clinical and radiological investigation using hydrostatic sialography. Clin Radiol 23:474–482

    Article  CAS  PubMed  Google Scholar 

  • Whaley K, Webb J, McAvory BA (1973a) Sjögren’s syndrome II. Clinical associations and immunological phenomena. Quart J Med 42:513–548

    CAS  PubMed  Google Scholar 

  • Whaley K, Williamson J, Chisholm DM et al (1973b) Sjögren’s syndrome I. Sicca components. Quart J Med 42:279–304

    CAS  PubMed  Google Scholar 

  • Williamson J, Alison M (1967) Effect of temperature and humidity in Schirmer’s tear test. Br J Opthal 51:596–598

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. 3rd Department of Rheumatology, National Institute of Rheumatology and Physiotherapy, Frankel Leó út 27-29, Budapest, 1023, Hungary

    Geza Balint

  2. Department of Medicine, McMaster University, Hamilton, ON, L8P 1H6, Canada

    W. Watson Buchanan & Walter Kean

  3. Haldimand War Memorial Hospital, 400 Broad Street, Dunnville, ON, N1A 2P7, Canada

    Colin A. Kean & Walter Kean

  4. Sheffield Hallam University, Sheffield, S1 1WB, UK

    K. D. Rainsford

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  1. Geza Balint
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Balint, G., Watson Buchanan, W., Kean, C.A. et al. Sjögren’s syndrome. Inflammopharmacol 32, 37–43 (2024). https://doi.org/10.1007/s10787-023-01222-z

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