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Review Article

Clinical practice guideline for Sjögren’s syndrome 2017

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Pages 383-408 | Received 31 Jan 2018, Accepted 03 Feb 2018, Published online: 13 Mar 2018

References

  • Tsuboi H, Hagiwara S, Asashima H, Takahashi H, Hirota T, Noma H, et al. Comparison of performance of the 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome with other sets of criteria in Japanese patients. Ann Rheum Dis. 2017;76:1980–5.
  • Vivino FB, Carsons SE, Foulks G, Daniels TE, Parke A, Brennan MT, et al. New treatment guidelines for Sjögren's disease. Rheum Dis Clin North Am. 2016;42:531–51.
  • Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, *British Society for Rheumatology Standards, Guideline and Audit Working Group, et al. The British Society for Rheumatology guideline for the management of adults with primary Sjögren's syndrome. Rheumatology (Oxford). 2017;56:e24–48.
  • Sumida T. Clinical practice guideline for Sjögren’s syndrome 2017 (Clinical practice guideline committee for Sjögren’s syndrome organized by the Research Team for Autoimmune Diseases, the Research Program for Intractable Disease of the Ministry of Health, Labor and Welfare). Tokyo: Shindantochiryousha; 2017. [In Japanese]
  • Fukui T, Yamaguchi N, Morizane T, Yoshida M, Kojimahara N. Medical Information Network Distribution Service (Minds) the guidance for development of clinical practice guideline 2014. Tokyo: Igakushoin; 2014 [In Japanese]
  • Gotoh S, Watanabe Y, Takeda M, Tomitsuka S. The chewing gum test: a sialometric evaluation for diagnosing Sjögren's syndrome. J Jpn Oral Muco Membr. 2002;8:20–8.
  • Gotoh S. Dry mouth and Sjögren's syndrome diagnosed by salivary volume-using gum test and unstimulated saliva. Iyaku J. 2003;39:2366–9.
  • Nakamura H, Kawakami A, Iwamoto N, Okada A, Yamasaki S, Tamai M, et al. A single centre retrospective analysis of AECG classification criteria for primary Sjögren's syndrome based on 112 minor salivary gland biopsies in a Japanese population. Rheumatology (Oxford). 2010;49:1290–3.
  • Yazisiz V, Avci AB, Erbasan F, Kiriş E, Terzioğlu E. Diagnostic performance of minor salivary gland biopsy, serological and clinical data in Sjögren's syndrome: a retrospective analysis. Rheumatol Int. 2009;29:303–409.
  • Gotoh S, Watanabe Y, Fujibayashi T. Validity of stimulated whole saliva collection as a sialometric evaluation for diagnosing Sjögren's syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;99:299–302.
  • Risselada AP, Kruize AA, Goldschmeding R, Lafeber FP, Bijlsma JW, van Roon JA. The prognostic value of routinely performed minor salivary gland assessments in primary Sjögren's syndrome. Ann Rheum Dis. 2014;73:1537–40.
  • Lida Santiago M, Seisdedos MR, García Salinas RN, Secco A, Marino Claverie L, Techera L, et al. Frequency of complications and usefulness of the minor salivary gland biopsy. Reumatol Clin. 2012;8:255–8.
  • Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Sjögren's International Collaborative Clinical Alliance (SICCA) Research Groups, et al. American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken). 2012;64:475–87.
  • Teppo H, Revonta M. A follow-up study of minimally invasive lip biopsy in the diagnosis of Sjögren's syndrome. Clin Rheumatol. 2007;26:1099–103.
  • Pijpe J, Kalk WW, van der Wal JE, Vissink A, Kluin PM, Roodenburg JL, et al. Parotid gland biopsy compared with labial biopsy in the diagnosis of patients with primary Sjögren's syndrome. Rheumatology (Oxford). 2007;46:335–41.
  • Rosas J, Ramos-Casals M, Ena J, García-Carrasco M, Verdu J, Cervera R, et al. Usefulness of basal and pilocarpine-stimulated salivary flow in primary Sjögren's syndrome. Correlation with clinical, immunological and histological features. Rheumatology (Oxford). 2002;41:670–5.
  • McGuirt WF Jr, Whang C, Moreland W. The role of parotid biopsy in the diagnosis of pediatric Sjögren syndrome. Arch Otolaryngol Head Neck Surg. 2002;128:1279–81.
  • Versura P, Frigato M, Cellini M, Mulè R, Malavolta N, Campos EC. Diagnostic performance of tear function tests in Sjogren's syndrome patients. Eye (London). 2007;21:229–37.
  • Mizuno Y, Yamada M, Miyake Y. Dry Eye Survey Group of the National Hospital Organization of Japan. Association between clinical diagnostic tests and health-related quality of life surveys in patients with dry eye syndrome. Jpn J Ophthalmol. 2010;54:259–65.
  • Wakamatsu TH, Sato EA, Matsumoto Y, Ibrahim OM, Dogru M, Kaido M, et al. Conjunctival in vivo confocal scanning laser microscopy in patients with Sjögren syndrome. Invest Ophthalmol Vis Sci. 2010;51:144–50.
  • Whitcher JP, Shiboski CH, Shiboski SC, Heidenreich AM, Kitagawa K, Zhang S, Sjögren's International Collaborative Clinical Alliance Research Groups, et al. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry. Am J Ophthalmol. 2010;149:305–415.
  • Qiu X, Gong L, Lu Y, Jin H, Robitaille M. The diagnostic significance of Fourier-domain optical coherence tomography in Sjögren syndrome, aqueous tear deficiency and lipid tear deficiency patients. Acta Ophthalmol. 2012;90:e359–66.
  • Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2016;55:450–60.
  • Ramos-Casals M, Brito-Zerón P, Seror R, Bootsma H, Bowman SJ, Dörner T, EULAR Sjögren Syndrome Task Force, et al. Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford). 2015;54:2230–8.
  • Gono T, Kawaguchi Y, Katsumata Y, Takagi K, Tochimoto A, Baba S, et al. Clinical manifestations of neurological involvement in primary Sjögren’s syndrome. Clin Rheumatol. 2011;30:485–90.
  • Morreale M, Marchione P, Giacomini P, Pontecorvo S, Marianetti M, Vento C, et al. Neurological involvement in primary Sjögren syndrome: a focus on central nervous system. PLoS One. 2014;9:e84605.
  • Retamozo S, Akasbi M, Brito-Zeron P, Bosch X, Bove A, Perez-de-Lis M, et al. Anti-Ro52 antibody testing influences the classification and clinical characterization of primary Sjögren's syndrome. Clin Exp Rheumatol. 2012;30:686–92.
  • Huo AP, Lin KC, Chou CT. Predictive and prognostic value of antinuclear antibodies and rheumatoid factor in primary Sjögren's syndrome. Int J Rheum Dis. 2010;13:3937.
  • Kessel A, Toubi E, Rozenbaum M, Zisman D, Sabo E, Rosner I. Sjögren's syndrome in the community: can serology replace salivary gland biopsy? Rheumatol Int. 2006;26:337–9.
  • Goëb V, Salle V, Duhaut P, Jouen F, Smail A, Ducroix JP, et al. Clinical significance of autoantibodies recognizing Sjögren's syndrome A (SSA), SSB, calpastatin and alpha-fodrin in primary Sjögren's syndrome. Clin Exp Immunol. 2007;148:281–7.
  • Gottenberg JE, Mignot S, Nicaise-Rolland P, Cohen-Solal J, Aucouturier F, Goetz J, et al. Prevalence of anti-cyclic citrullinated peptide and anti-keratin antibodies in patients with primary Sjögren's syndrome. Ann Rheum Dis. 2005;64:114–7.
  • Bournia VK, Diamanti KD, Viachoyiannopoulos PG, Moutsopoulos HM. Anticentromere antibody positive Sjögren's syndrome: a retrospective descriptive analysis. Arthritis Res Ther. 2010;12:R47.
  • Kitagawa T, Shibasaki K, Toya S. Clinical significance and diagnostic usefulness of anti-centromere antibody in Sjögren's syndrome. Clin Rheumatol. 2012;31:105–12.
  • Chen KS, Jiang MC, Li CJ, Liu OK, Tsai CS. Discrimination between Sjögren's syndrome and non-Sjögren's sicca syndrome by sialoscintigraphy and antibodies against alpha-fodrin and Ro/La autoantigens. J Int Med Res. 2009;37:1088–96.
  • Kim SM, Park E, Lee JH, Lee SH, Kim HR. The clinical significance of anti-cyclic citrullinated peptide antibody in primary Sjögren syndrome. Rheumatol Int. 2012;32:3963–7.
  • Mori K, Iida S, Shimada J, Yoshida A, Tamura N, Tamura N, et al. Clinical study of the usefulness of anti-SS-A/Ro antibody, anti-SS-B/La antibody, and the salivary test in diagnosing Sjogren's syndrome. J Meikai Dental Med. 2009;38:67–70. [Article in Japanese]
  • Takada K, Suzuki K, Matsumoto M, Okada M, Nakanishi T, Horikoshi H, et al. The relationships between titers of anti-Ro or anti-La as measured by ELISA and salivary production rate with age correction. Mod Rheumatol. 2008;18:578–84.
  • Aoki A, Kirino Y, Ishigatsubo Y, Senuma A, Nagaoka S. Liver involvement in primary Sjögren's syndrome. Jpn J Clin Immunol. 2004;27:397–401.
  • Aoki A, Ohno S, Ueda A, Ideguchi H, Ohkubo T, Hagiwara E, et al. Hematological abnormalities of primary Sjögren's syndrome. Nihon Rinsho Meneki Gakkai Kaishi. 2000;2:124–8.
  • Ramos-Casals M, García-Carrasco M, Cervera R, Gaya J, Halperin I, Ubieto I, et al. Thyroid disease in primary Sjögren's syndrome. Study in a series of 160 patients. Medicine (Bortimore). 2000;79:103–8.
  • Caramaschi P, Biasi D, Caimmi C, Scambi C, Pieropan S, Barausse G, et al. The co-occurrence of Hashimoto thyroiditis in primary Sjogren's syndrome defines a subset of patients with milder clinical phenotype. Rheumatol Int. 2013;33:1271–5.
  • Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A. A longitudinal cohort study of Finnish patients with primary Sjögren's syndrome: clinical, immunological and epidemiological aspects. Ann Rheum Dis. 2001;60:467–72.
  • Yang Y, Li Z, Wang L, Zhang F. The clinical and laboratory characteristics of Sjögren's syndrome that progresses to systemic lupus erythematosus: a retrospective case–control study. Int J Rheum Dis. 2013;16:173–7.
  • Takagi Y, Kimura Y, Nakamura H, Sasaki M, Eguchi K, Nakamura T. Salivary gland ultrasonography: can it be an alternative to sialography as an imaging modality for Sjogren's syndrome? Ann Rheum Dis. 2010;69:1321–4.
  • Takagi Y, Sumi M, Nakamura H, Sato S, Kawakami A, Nakamura T. Salivary gland ultrasonography as a primary imaging tool for predicting efficacy of xerostomia treatment in patients with Sjögren's syndrome. Rheumatology (Oxford). 2016;55:237–45.
  • Takagi Y, Sumi M, Van Cauteren M, Nakamura T. Fast and high-resolution MR sialography using a small surface coil. J Magn Reson Imaging. 2005;22:29–37.
  • Takagi Y, Sumi M, Sumi T, Ichikawa Y, Nakamura T. MR microscopy of the parotid glands in patients with Sjogren's syndrome: quantitative MR diagnostic criteria. AJNR Am J Neuroradiol. 2005;26:1207–14.
  • El Miedany YM, Ahmed I, Mourad HG, Mehanna AN, Aty SA, Gamal HM, et al. Quantitative ultrasonography and magnetic resonance imaging of the parotid gland: can they replace the histopathologic studies in patients with Sjogren's syndrome? Joint Bone Spine. 2004;71:29–38.
  • Izumi M, Hida A, Takagi Y, Kawabe Y, Eguchi K, Nakamura T. MR imaging of the salivary glands in Sicca syndrome: comparison of lipid profiles and imaging in patients with hyperlipidemia and patients with Sjögren’s syndrome. AJR Am J Roentgenol. 2000;175:829–34.
  • Shizukuishi K, Nagaoka S, Kinno Y, Saito M, Takahashi N, Kawamoto M, et al. Scoring analysis of salivary gland scintigraphy in patients with Sjögren's syndrome. Ann Nucl Med. 2003;17:627–31.
  • Booker J, Howarth D, Taylor L, Voutnis D, Sutherland D. Appropriate utilization of semi-quantitative analysis in salivary scintigraphy. Nucl Med Commun. 2004;25:1203–10.
  • Tonami H, Higashi K, Matoba M, Yokota H, Yamamoto I, Sugai S. A comparative study between MR sialography and salivary gland scintigraphy in the diagnosis of Sjögren syndrome. J Comput Assist Tomogr. 2001;25:262–8.
  • Kim HA, Yoon SH, Yoon JK, Lee SJ, Jo KS, Lee DH, et al. Salivary gland scintigraphy in Sjögren's syndrome. Comparison of the diagnostic performance of visual and semiquantitative analysis. Nuklearmedizin. 2014;53:139–45.
  • Aung W, Murata Y, Ishida R, Takahashi Y, Okada N, Shibuya H. Study of quantitative oral radioactivity in salivary gland scintigraphy and determination of the clinical stage of Sjögren’s syndrome. J Nucl Med. 2001;42:38–43.
  • Dugonjić S, Stefanović D, Ethurović B, Spasić-Jokić V, Ajdinović B. Evaluation of diagnostic parameters from parotid and submandibular dynamic salivary glands scintigraphy and unstimulated sialometry in Sjögren’s syndrome. Hell J Nucl Med. 2014;17:116–22.
  • Tomiita M, Ueda T, Nagata H, Tanabe E, Shimojo N, Saito K, et al. Usefulness of magnetic resonance sialography in patients with juvenile Sjögren’s syndrome. Clin Exp Rheumatol. 2005;23:540–4.
  • Chikui T, Shimizu M, Kawazu T, Okamura K, Shiraishi T, Yoshiura K. A quantitative analysis of sonographic images of the salivary gland: a comparison between sonographic and sialographic findings. Ultrasound Med Biol. 2009;35:1257–64.
  • Milic VD, Petrovic RR, Boricic IV, Radunovic GL, Pejnovic NN, Soldatovic I, et al. Major salivary gland sonography in Sjögren's syndrome: diagnostic value of a novel ultrasonography score (0–12) for parenchymal inhomogeneity. Scand J Rheumatol. 2010;39:160–6.
  • Takeda M, Fujibayashi T, Okazaki M, Shibusawa F, Gotoh S, Watanabe Y. MR sialography in the diagnosis of Sjögren’s syndrome – comparative study with other clinical examinations. J Jpn Oral Muco Membr. 2003;9:56–64. [Article in Japanese]
  • Ramos-Casals M, Brito-Zerón P, Perez DE, Lis M, et al. Clinical and prognostic significance of parotid scintigraphy in 405 patients with primary Sjögren’s syndrome. J Rheumatol. 2010;37:585–90.
  • Zou Q, Jiao J, Zou MH, Xu JH, Pan YF, Chen JN, et al. Semi-quantitative evaluation of salivary gland function in Sjögren’s syndrome using salivary gland scintigraphy. Clin Rheumatol. 2012;31:1699–705.
  • Kalk WW, Vissink A, Spijkervet FK, Möller JM, Roodenburg JL. Morbidity from parotid sialography. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;92:572–5.
  • Shimizu M, Okamura K, Yoshiura K, Ohyama Y, Nakamura S, Kinukawa N. Sonographic diagnostic criteria for screening Sjögren's syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;102:85–93.
  • Ohbayashi N. Diagnosis of Sjögren’s syndrome using MR sialography. Dental Radiol. 2004;44:43–8. [Article in Japanese]
  • Takagi Y, Sumi M, Nakamura H, Iwamoto N, Horai Y, Kawakami A, et al. Ultrasonography as an additional item in the American College of Rheumatology classification of Sjögren’s syndrome. Rheumatology (Oxford). 2014;53:1977–83.
  • Voulgarelis M, Ziakas PD, Papageorgiou A, Baimpa E, Tzioufas AG, Moutsopoulos HM. Prognosis and outcome of non-Hodgkin lymphoma in primary Sjögren syndrome. Medicine (Baltimore). 2012;91:1–9.
  • Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65:796–803.
  • Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome. Arthritis Rheum. 2002;46:741–7.
  • Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI), et al. Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis. 2016;75:348–55.
  • Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA. Epidemiology of primary Sjögren's syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford). 2006;45:187–91.
  • Pollard RP, Pijpe J, Bootsma H, Spijkervet FK, Kluin PM, Roodenburg JL, et al. Treatment of mucosa-associated lymphoid tissue lymphoma in Sjogren's syndrome: a retrospective clinical study. J Rheumatol. 2011;38:2198–208.
  • Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM. Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects. Medicine (Baltimore). 2009;88:284–93.
  • Ekström SK, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J, et al. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. Blood. 2008;111:40293038.
  • Papageorgiou A, Ziogas DC, Mavragani CP, Zintzaras E, Tzioufas AG, Moutsopoulos HM, et al. Predicting the outcome of Sjogren's syndrome-associated non-Hodgkin's lymphoma patients. PLoS One. 2015;10:e0116189.
  • Anderson LA, Gadalla S, Morton LM, Landgren O, Pfeiffer R, Warren JL, et al. Population-based study of autoimmune conditions and the risk of specific lymphoid malignancies. Int J Cancer. 2009;125:398–405.
  • Brito-Zerón P, Ramos-Casals M, Bove A, Sentis J, Font J. Predicting adverse outcomes in primary Sjogren's syndrome: identification of prognostic factors. Rheumatology (Oxford). 2007;46:1359–62.
  • Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren's syndrome. Semin Arthritis Rheum. 2000;29:296–304.
  • Baldini C, Pepe P, Luciano N, Ferro F, Talarico R, Grossi S, et al. A clinical prediction rule for lymphoma development in primary Sjögren's syndrome. J Rheumatol. 2012;39:804–8.
  • Ramos-Casals M, Brito-Zerón P, Yagüe J, Akasbi M, Bautista R, Ruano M, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren's syndrome. Rheumatology (Oxford). 2005;44:89–93.
  • Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome. Arthritis Rheum. 1999;42:1765–72.
  • Sutcliffe N, Inanc M, Speight P, Isenberg D. Predictors of lymphoma development in primary Sjögren's syndrome. Semin Arthritis Rheum. 1998;28:80–7.
  • Cimaz R, Casadei A, Rose C, Bartunkova J, Sediva A, Falcini F, et al. Primary Sjögren syndrome in the paediatric age: a multicentre survey. Eur J Pediatr. 2003;162:661–5.
  • Saad Magalhães C, de Souza Medeiros PB, Oliveira-Sato J, Custódio-Domingues MA. Clinical presentation and salivary gland histopathology of paediatric primary Sjo¨gren’s syndrome. Clin Exp Rheumatol. 2011;29:589–93.
  • Schuetz C, Prieur AM, Quartier P. Sicca syndrome and salivary gland infiltration in children with autoimmune disorders: when can we diagnose Sjo¨gren’s syndorome? Clin Exp Rheumatol. 2010;28:434–9.
  • Singer NG, Tomanova-Soltys I, Lowe R. Sjögren's syndrome in childhood. Curr Rheumatol Rep. 2008;10:147–55.
  • Houghton K, Malleson P, Cabral D, Petty R, Tucker L. Primary Sjögren's syndrome in children and adolescents: are proposed diagnostic criteria applicable? J Rheumatol. 2005;32:2225–32.
  • Stiller M, Golder W, Do¨ring E, Biedermann T. Primary and secondary Sjo¨gren’s syndrome in children – a comparative study. Clin Oral Investig. 2000;4:176–82.
  • Takei S. [Sjögren's syndrome (SS) in childhood: is it essentially different from adult SS?]. Nihon Rinsho Meneki Gakkai Kaishi. 2010;33:8–14. [Article in Japanese]
  • Yokogawa N, Lieberman SM, Alawi F, Bout-Tabaku S, Guttenberg M, Sherry DD, et al. Comparison of labial minor salivary gland biopsies from childhood Sjögren syndrome and age-matched controls. J Rheumatol. 2014;41:1178–82.
  • Petrone D, Condemi JJ, Fife R, Gluck O, Cohen S, Dalgin P. A double-blind, randomized, placebo-controlled study of cevimeline in Sjögren's syndrome patients with xerostomia and keratoconjunctivitis sicca. Arthritis Rheum. 2002;46:748–54.
  • Leung KC, McMillan AS, Wong MC, Leung WK, Mok MY, Lau CS. The efficacy of cevimeline hydrochloride in the treatment of xerostomia in Sjögren's syndrome in southern Chinese patients: a randomised double-blind, placebo-controlled crossover study. Clin Rheumatol. 2008;27:429336.
  • Fife RS, Chase WF, Dore RK, Wiesenhutter CW, Lockhart PB, Tindall E, et al. Cevimeline for the treatment of xerostomia in patients with Sjögren's syndrome: a randomized trial. Arch Intern Med. 2002;162:1293–300.
  • Wu CH, Hsieh SC, Lee KL, Li KJ, Lu MC, Yu CL. Pilocarpine hydrochloride for the treatment of xerostomia in patients with Sjögren's syndrome in Taiwan – a double-blind, placebo-controlled trial. J Formos Med Assoc. 2006;105:796–803.
  • Papas AS, Sherrer YS, Charney M, Golden HE, Medsger TA Jr, Walsh BT, et al. Successful treatment of dry mouth and dry eye symptoms in Sjögren's syndrome patients with oral pilocarpine: a randomized, placebo-controlled, dose-adjustment study. J Clin Rheumatol. 2004;10:169–77.
  • Vivino FB, Al-Hashimi I, Khan Z, LeVeque FG, Salisbury PL, 3rd Tran-Johnson TK, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. Arch Intern Med. 1999;159:174–81.
  • Ohno S. The effect of Kampo medicine on salivary on salivary secretion in Sjögren's syndrome. Kampo Newest Therapy. 2006;15:134–40. [Article in Japanese]
  • Nishizawa Y, Nishizawa Y, Yoshioka F, Nosaka S, Yamada M, Yasuda R, et al. Long-term effect of traditional Chinese herbal medicine, mai-men-don-tang on sicca syndrome, especially, salicary secretion in patients with primary Sjogren’s syndrome: a multicenter, randomized well controlled group – pararell double-blinded study. J Jpn Saliv Gl. 2004;45:66–74. [Article in Japanese]
  • Torikai K, Natsume I, Okuda S, Miyasaka N, Nagai T. Clinical application of artificial saliva (Saliveht) in patients with Sjoegren's syndrome and the therapeutic results. Jpn J Med Pharm Sci. 1986;16:169–76. [Article in Japanese]
  • Chakraborty PP, Bhattacharjee R, Maiti AK, Mandal SK. Recurrent submandibular gland swelling as the initial presentation of Sjogren's syndrome. J Assoc Physicians India. 2007;55:44.
  • Sugimoto T, Uzu T, Kashiwagi A. Recurrent parotitis as a first manifestation of adult primary Sjögren's syndrome. Intern Med. 2006;45:831–2.
  • Alp H, Orbak Z, Erdogan T, Karabag K, Gursan N. Recurrent parotitis as a first manifestation in a child with primary Sjögren's syndrome. West Indian Med J. 2011;60:685–7.
  • Baszis K, Toib D, Cooper M, French A, White A. Recurrent parotitis as a presentation of primary pediatric Sjögren syndrome. Pediatrics. 2012;129:e179–82.
  • Civilibal M, Canpolat N, Yurt A, Kurugoglu S, Erdamar S, Bagci O, et al. A child with primary Sjögren syndrome and a review of the literature. Clin Pediatr (Phila). 2007;46:738–42.
  • Alves MB, Motta AC, Messina WC, Migliari DA. Saliva substitute in xerostomic patients with primary Sjögren's syndrome: a single-blind trial. Quintessence Int. 2004;35:392–6.
  • De Luca R, Trodella M, Vicidomini A, Colella G, Tartaro G. Endoscopic management of salivary gland obstructive diseases in patients with Sjögren's syndrome. J Craniomaxillofac Surg. 2015;43:1643–9.
  • Shacham R, Puterman MB, Ohana N, Nahlieli O. Endoscopic treatment of salivary glands affected by autoimmune diseases. J Oral Maxillofac Surg. 2011;69:376–481.
  • Iwao A, Sugiyama K. A case of Sjögren's syndrome with recurrent swelling of parotid glands. J Pediatr Pract. 2004;67:1351–4. [Article in Japanese]
  • Yasuda S, Ogura N, Horita T, Yasuda I, Hioka T, Kondo N, et al. Abacterial prostatitis and primary biliary cirrhosis with Sjogren's syndrome. Mod Rheumatol. 2004;14:70–2.
  • Miyazaki M, Suzuki M, Someya T, Fujii T, Takahashi K, Kinoshita K, et al. A case of Sjögren's syndrome in an 11-year old girl diagnosed by recurrent swelling of parotid glands. Jpn J Pediatr. 2003;56:337–41. [Article in Japanese]
  • Izumi M, Eguchi K, Nakamura H, Takagi Y, Kawabe Y, Nakamura T. Corticosteroid irrigation of parotid gland for treatment of xerostomia in patients with Sjögren's syndrome. Ann Rheum Dis. 1998;57:464–9.
  • Takagi Y, Katayama I, Tashiro S, Nakamura T. Parotid irrigation and cevimeline gargle for treatment of xerostomia in Sjögren's syndrome. J Rheumatol. 2008;35:2289–91.
  • Koh S, Ikeda C, Takai Y, Watanabe H, Maeda N, Nishida K. Long-term results of treatment with diquafosol ophthalmic solution for aqueous-deficient dry eye. Jpn J Ophthalmol. 2013;57:440–6.
  • McDonald CC, Kaye SB, Figueiredo FC, Macintosh G, Lockett C. A randomised, crossover, multicentre study to compare the performance of 0.1% (w/v) sodium hyaluronate with 1.4% (w/v) polyvinyl alcohol in the alleviation of symptoms associated with dry eye syndrome. Eye (Lond). 2002;16:601–7.
  • Arimoto A, Kitagawa K, Mita N, Takahashi Y, Shibuya E, Sasaki H. Effect of rebamipide ophthalmic suspension on signs and symptoms of keratoconjunctivitis sicca in Sjögren syndrome patients with or without punctal occlusions. Cornea. 2014;33:806–11.
  • Koh S, Maeda N, Ikeda C, Oie Y, Soma T, Tsujikawa M, et al. Effect of diquafosol ophthalmic solution on the optical quality of the eyes in patients with aqueous-deficient dry eye. Acta Ophthalmol. 2014;92:e671–5.
  • Qiu W, Liu Z, Ao M, Li X, Wang W. Punctal plugs versus artificial tears fortreating primary Sjögren's syndrome with keratoconjunctivitis SICCA: a comparative observation of their effects on visual function. Rheumatol Int. 2013;33:2543–8.
  • Fox PC, Datiles M, Atkinson JC, Macynski AA, Scott J, Fletcher D, et al. Prednisone and piroxicam for treatment of primary Sjögren's syndrome. Clin Exp Rheumatol. 1993;11:149–56.
  • Miyawaki S, Nishiyama S, Matoba K. Efficacy of low-dose prednisolone maintenance for saliva production and serological abnormalities in patients with primary Sjögren's syndrome. Intern Med. 1999;38:938–3.
  • Reksten TR, Brokstad KA, Jonsson R, Brun JG, Jonsson MV. Implications of long-term medication of oral steroids and antimalarial drugs in primary Sjögren's syndrome. Int Immunopharmacol. 2011;11:2125–9.
  • Li H, Liu Z, Gong Y, Jiang Z, Zhang Y, Dai Y, et al. Application of immunosuppressant facilitates the therapy of optic neuritis combined with Sjögren's syndrome. Chin Med J. 2014;127:3098–104.
  • Ichikawa Y, Yamauchi K, Shimizu Y, Arimori S. Effect of steroid hormone on the salivary and lacrimal secretions in Sjögren's syndrome. Ryumachi. 1979;19:15–22. [Article in Japanese]
  • Maripuri S, Grande JP, Osborn TG, Fervenza FC, Matteson EL, Donadio JV, et al. Renal involvement in primary Sjögren's syndrome: a clinicopathologic study. Clin J Am Soc Nephrol. 2009;4:1423–31.
  • Katayama I, Kotobuki Y, Kiyohara E, Murota H. Annular erythema associated with Sjögren's syndrome: review of the literature on the management and clinical analysis of skin lesions. Mod Rheumatol. 2010;20:123–9.
  • Shi JH, Liu HR, Xu WB, Feng RE, Zhang ZH, Tian XL, et al. Pulmonary manifestations of Sjögren's syndrome. Respiration. 2009;78:377–86.
  • Drosos AA, Skopouli FN, Costopoulos JS, Papadimitriou CS, Moutsopoulos HM. Cyclosporin A (CyA) in primary Sjögren's syndrome: a double blind study. Ann Rheum Dis. 1986;45:732–5.
  • Price EJ, Rigby SP, Clancy U, Venables PJ. A double blind placebo controlled trial of azathioprine in the treatment of primary Sjögren's syndrome. J Rheumatol. 1998;5:896–9.
  • Nakayamada S, Fujimoto T, Nonomura A, Saito K, Nakamura S, Tanaka Y. Usefulness of initial histological features for stratifying Sjogren's syndrome responders to mizoribine therapy. Rheumatology (Oxford). 2009;48:1279–82.
  • Nakayamada S, Saito K, Umehara H, Ogawa N, Sumida T, Ito S, et al. Efficacy and safety of mizoribine for the treatment of Sjögren's syndrome: a multicenter open-label clinical trial. Mod Rheumatol. 2007;17:464–9.
  • Skopouli FN, Jagiello P, Tsifetaki N, Moutsopoulos HM. Methotrexate in primary Sjögren's syndrome. Clin Exp Rheumatol. 1996;14:555–8.
  • Font J, Ramos-Casals M, de la Red G, Pou A, Casanova A, García-Carrasco M, et al. Pure sensory neuropathy in primary Sjögren's syndrome. Longterm prospective followup and review of the literature. J Rheuatol. 2003;30:1552–7.
  • de Seze J, Delalande S, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Myelopathies secondary to Sjögren's syndrome: treatment with monthly intravenous cyclophosphamide associated with corticosteroids. J Rheumatol. 2006;33:709–11.
  • Mariette X, Ravaud P, Steinfeld S, Baron G, Goetz J, Hachulla E, et al. Inefficacy of infliximab in primary Sjögren's syndrome: results of the randomized, controlled Trial of Remicade in Primary Sjögren's Syndrome (TRIPSS). Arthritis Rheum. 2004;50:1270–6.
  • Steinfeld SD, Demols P, Appelboom T. Infliximab in primary Sjögren's syndrome: one-year followup. Arthritis Rheum. 2002;46:3301–3.
  • Steinfeld SD, Demols P, Salmon I, Kiss R, Appelboom T. Infliximab in patients with primary Sjögren's syndrome: a pilot study. Arthritis Rheum. 2001;44:2371–5.
  • Sankar V, Brennan MT, Kok MR, Leakan RA, Smith JA, Manny J, et al. Etanercept in Sjören's syndrome: a twelve-week randomized, double-blind, placebo-controlled pilot clinical trial. Arthritis Rheum. 2004;50:2240–5.
  • Zandbelt MM, de Wilde P, van Damme P, Hoyng CB, van de Putte L, van den Hoogen F. Etanercept in the treatment of patients with primary Sjögren's syndrome: a pilot study. J Rheumatol. 2004;31:96–101.
  • Tsuboi H, Matsumoto I, Hagiwara S, Hirota T, Takahashi H, Ebe H, et al. Efficacy and safety of abatacept for patients with Sjögren's syndrome associated with rheumatoid arthritis: rheumatoid arthritis with orencia trial toward Sjögren's syndrome Endocrinopathy (ROSE) trial-an open-label, one-year, prospective study-Interim analysis of 32 patients for 24 weeks. Mod Rheumatol. 2015;25:187–93.
  • Meiners PM, Vissink A, Kroese FG, Spijkervet FK, Smitt-Kamminga NS, Abdulahad WH, et al. Abatacept treatment reduces disease activity in early primary Sjögren's syndrome (open-label proof of concept ASAP study). Ann Rheum Dis. 2014;73:1393–6.
  • Meijer JM, Meiners PM, Vissink A, Spijkervet FK, Abdulahad W, Kamminga N, et al. Effectiveness of rituximab treatment in primary Sjögren's syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2010;62:960–8.
  • Devauchelle-Pensec V, Mariette X, Jousse-Joulin S, Berthelot JM, Perdriger A, Puéchal X, et al. Treatment of primary Sjögren syndrome with rituximab: a randomized trial. Ann Intern Med. 2014;160:233–42.
  • Dass S, Bowman SJ, Vital EM, Ikeda K, Pease CT, Hamburger J, et al. Reduction of fatigue in Sjögren syndrome with rituximab: results of a randomised, double-blind, placebo-controlled pilot study. Ann Rheum Dis. 2008;67:1541–4.
  • Carubbi F, Cipriani P, Marrelli A, Benedetto P, Ruscitti P, Berardicurti O, et al. Efficacy and safety of rituximab treatment in early primary Sjögren's syndrome: a prospective, multi-center, follow-up study. Arthritis Res Ther. 2013;15:R172.
  • Pijpe J, van Imhoff GW, Spijkervet FK, Roodenburg JL, Wolbink GJ, Mansour K, et al. Rituximab treatment in patients with primary Sjögren's syndrome: an open-label phase II study. Arthritis Rheum. 2005;52:2740–50.
  • St Clair EW, Levesque MC, Prak ET, Vivino FB, Alappatt CJ, Spychala ME, Autoimmunity Centers of Excellence, et al. Rituximab therapy for primary Sjögren's syndrome: an open-label clinical trial and mechanistic analysis. Arthritis Rheum. 2013;65:1097–106.
  • Mariette X, Seror R, Quartuccio L, Baron G, Salvin S, Fabris M, et al. Efficacy and safety of belimumab in primary Sjögren's syndrome: results of the BELISS open-label phase II study. Ann Rheum Dis. 2015;74:526–31.
  • Gottenberg JE, Cinquetti G, Larroche C, Combe B, Hachulla E, Meyer O, Club Rhumatismes et Inflammations and the French Society of Rheumatology, et al. Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis. 2013;72:1026–31.
  • Mekinian A, Ravaud P, Larroche C, Hachulla E, Gombert B, Blanchard-Delaunay C, *Club Rhumatismes et Inflammation, et al. Rituximab in central nervous system manifestations of patients with primary Sjögren's syndrome: results from the AIR registry. Clin Exp Rheumatol. 2012;30:208–12.
  • Devauchelle-Pensec V, Morvan J, Rat AC, Jousse-Joulin S, Pennec Y, Pers JO, et al. Effects of rituximab therapy on quality of life in patients with primary Sjögren's syndrome. Clin Exp Rheumatol. 2011;29:6–12.
  • Adler S, Körner M, Förger F, Huscher D, Caversaccio MD, Villiger PM. Evaluation of histologic, serologic, and clinical changes in response to abatacept treatment of primary Sjögren’s syndrome: a pilot study. Arthritis Care Res (Hoboken). 2013;65:1862–8.
  • Iwata N, Miyamae T, Kikuchi M, Kishi T, Hara R, Kaneko U, et al. Clinical and histological experience of labial lip biopsy in juvenile Sjögren's syndrome. Nihon Rinsho Meneki Gakkai Kaishi. 2009;32:195–200.
  • Shikano T. Childhood Sjögren's syndrome having aseptic meningitis and hemophagocytic-like syndrome. Nihon Shoni Ketueki Gakkai Zasshi. 2002;16:21–6.
  • Hoshina T, Ymaguchi Y, Ohga S, Kira R, Ishimura M, Takada H, et al. Sjogren's syndrome-associated meningoencephalomyelitis: cerebrospinal fluid cytokine levels and therapeutic utility of tacrolimus. J Neurol Sci. 2008;267:182–6.
  • Bogdanović R, Basta-Jovanović G, Putnik J, Stajić N, Paripović A. Renal involvement in primary Sjogren syndrome of childhood: case report and literature review. Mod Rheumatol. 2013;23:182–9.
  • Shikano T, Harumi K, Akemi K. An 11-year-old male of Sjögren's syndrome with meningitis, myositis and DIC. Jpn J Pediatr. 1999;52:2125–9. [Article in Japanese]
  • Kobayashi I, Furuta H, Tame A, Kawamura N, Kojima K, Endoh M, et al. Complications of childhood Sjögren's syndrome. Eur J Pediatr. 1996;155:890–3.
  • Ohtsuka T, Saito Y, Hasegawa M, Tatsuno M, Takita S, Arita M, et al. Central nervous system disease in a child with primary Sjögren syndrome. J Pediatr. 1995;127:961–3.
  • Matsunaga T, Akazawa H, Nakajima M, Shima M, Yoshioka A. A case of primary Sjögren's syndrome manifesting aseptic meningoencephalitis. Jpn J Pediatr. 1995;99:2011–5. [Article in Japanese]
  • Kosuga M, Kimura K, Okabe I, Fukami S, Miura T. Childhood primary Sjögren syndrome having aseptic meningitis. Jpn J Pediatr. 1998;51:953–6. [Article in Japanese]
  • Ohnishi T, Urushihara M, Kondo S, Koumi S, Kuroda Y. A girl diagnosed as primary Sjögren syndrome by recurrent aseptic meningitis. Jpn J Pediatr. 2000;92:104. [Article in Japanese]
  • Yasoshima K, Nawate M, Mino E, Isizaki S, Isizaka J. A girl diagnosed as Sjögren syndrome with aseptic meningitis. Jpn J Pediatr. 1999;47:187–8. [Article in Japanese]
  • Sekiguchi A. A case of a childhood Sjögren syndrome. Clin Dermatol. 2010;54:1383–6. [Article in Japanese]
  • Tomiita M. Pediatric Sjögren's syndrome. Inflamm Immunity. 2013;21:167–72. [Article in Japanese]
  • Tomiita M, Takei S, Kuwada N, Nonaka Y, Saito K, Shimojo N, et al. Efficacy and safety of orally administered pilocarpine hydrochloride for patients with juvenile-onset Sjögren's syndrome. Mod Rheumatol. 2010;20:486–90.
  • de Souza TR, Silva IH, Carvalho AT, Gomes VB, Duarte AP, Leão JC, et al. Juvenile Sjögren’s syndrome: distinctive age, unique findings. Pediatr Dent. 2012;34:427–30.
  • Eliasson H, Sonesson SE, Sharland G, Granath F, Simpson JM, Carvalho JS, et al. Working Group of the European Association of Pediatric Cardiology. Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients. Circulation. 2011;124:1919–26. Am J Cardiol 2009;103:1102–106.
  • Friedman DM, Kim MY, Copel JA, Llanos C, Davis C, Buyon JP. Prospective evaluation of fetuses with autoimmune-associated congenital heart block followed in the PR Interval and Dexamethasone Evaluation (PRIDE) Study. Am J Cardiol. 2009;103:1102–6.
  • Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation. 2004;110:1542–8.
  • Tsuboi H, Sumida T, Noma H, Yamagishi K, Anami A, Fukushima K, et al. Maternal predictive factors for fetal congenital heart block in pregnant mothers positive for anti-SS-A antibodies. Mod Rheumatol. 2016;26:569–75.
  • Costedoat-Chalumeau N, Amoura Z, Le Thi Hong D, Wechsler B, Vauthier D, Ghillani P, et al. Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block. Ann Rheum Dis. 2003;62:1010–2.
  • Shinohara K, Miyagawa S, Fujita T, Aono T, Kidoguchi K. Neonatal lupus erythematosus: results of maternal corticosteroid therapy. Obstet Gynecol. 1999;93:952–7.
  • Pisoni CN, Brucato A, Ruffatti A, Espinosa G, Cervera R, Belmonte-Serrano M, et al. Failure of intravenous immunoglobulin to prevent congenital heart block: findings of a multicenter, prospective, observational study. Arthritis Rheum. 2010;62:1147–52.
  • Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, Khamashta MA, Kim MY, Saxena A, et al. Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus. Circulation. 2012;126:76–82.
  • Gleicher N, Elkayam U. Preventing congenital neonatal heart block in offspring of mothers with anti-SSA/Ro and SSB/La antibodies: a review of published literature and registered clinical trials. Autoimmun Rev. 2013;12:1039–45.
  • Van Bergen AH, Cuneo BF, Davis N. Prospective echocardiographic evaluation of atrioventricular conduction in fetuses with maternal Sjögren's antibodies. Am J Obstet Gynecol. 2004;191:1014–8.
  • Krishnan A, Arya B, Moak JP, Donofrio MT. Outcomes of fetal echocardiographic surveillance in anti-SSA exposed fetuses at a large fetal cardiology center. Prenat Diagn. 2014;34:1207–12.
  • Gladman G, Silverman ED, Yuk-Law Luy L, Boutin C, Laskin C, Smallhorn JF. Fetal echocardiographic screening of pregnancies of mothers with anti-Ro and/or anti-La antibodies. Am J Perinatol. 2002;19:73–80.
  • Costedoat-Chalumeau N, Amoura Z, Lupoglazoff JM, Huong DL, Denjoy I, Vauthier D, et al. Outcome of pregnancies in patients with anti-SSA/Ro antibodies: a study of 165 pregnancies, with special focus on electrocardiographic variations in the children and comparison with a control group. Arthritis Rheum. 2004;50:3187–93.
  • Jaeggi ET, Silverman ED, Laskin C, Kingdom J, Golding F, Weber R. Prolongation of the atrioventricular conduction in fetuses exposed to maternal anti-Ro/SSA and anti-La/SSB antibodies did not predict progressive heart block. A prospective observational study on the effects of maternal antibodies on 165 fetuses. J Am Coll Cardiol. 2011;57:1487–92.
  • Friedman DM, Kim MY, Copel JA, Davis C, Phoon CK, Glickstein JS, et al. PRIDE Investigators. Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study. Circulation. 2008;117:485–93.