Overview
Sjögren's syndrome is a chronic autoimmune disorder characterized by exocrine gland dysfunction, primarily affecting salivary and lacrimal glands, leading to symptoms such as dry mouth and eyes. It can also involve multiple organ systems, including neurological and hematological manifestations. 13618Diagnosis
Management
Special Populations
Key Recommendations
References
Showing 100 most recent of 116 indexed papers.
1 Togizbayev G, Aubakirova B, Dilmanova D, Zaripova L, Tabenova A, Karina K et al.. 2024 Comprehensive Recommendations of the Qazaq College of Rheumatology for the Diagnosis and Management of Sjögren's Syndrome. International journal of rheumatic diseases 2025. link 2 Zheng X, Di J, Chen X, Li F, Liu Y, Cao J et al.. Meta-analysis of the efficacy and safety of rituximab in the treatment of primary Sjögren's syndrome. Frontiers in immunology 2025. link 3 de Oliveira FR, Appenzeller S, Pasoto SG, Fernandes MLMS, Lemos Lopes ML, de Magalhães Souza Fialho SC et al.. Recommendations on neurologic, cognitive, and psychiatric manifestations in patients with Sjögren's disease by the Brazilian Society of Rheumatology. Advances in rheumatology (London, England) 2025. link 4 Wu KY, Dave A, Nirwal GK, Giunta M, Nguyen VDH, Tran SD. Exosome Innovations in Ophthalmology and Sjögren's Syndrome. Advances in experimental medicine and biology 2025. link 5 Wu J, Li D. Pilocarpine treatment of Sjögren's syndrome curative effect of meta-analysis of randomised controlled trials. Clinical and experimental rheumatology 2024. link 6 Pugliesi A, Egypto DCSD, Duffles G, Cantali DU, Pasoto SG, Oliveira FR et al.. Recommendations on cutaneous and hematological manifestations of Sjögren's disease by the Brazilian Society of Rheumatology. Advances in rheumatology (London, England) 2024. link 7 Khavandgar Z, Warner BM, Baer AN. Evaluation and management of dry mouth and its complications in rheumatology practice. Expert review of clinical immunology 2024. link 8 Shindo T, Ito M, Sugiyama T, Okuyama T, Kono M, Atsumi T et al.. Diagnostic Value of Vessel Wall Imaging to Determine the Timing of Extracranial‒Intracranial Bypass for Moyamoya Syndrome Associated with Active Sjögren's Syndrome: A Case Report. Journal of neurological surgery. Part A, Central European neurosurgery 2024. link 9 Zhang A, Chen S, Lin R. Combined use of total glucosides of paeony and hydroxychloroquine in primary Sjögren's syndrome: A systematic review. Immunity, inflammation and disease 2023. link 10 Zheng A, Hu N, Xu J, Yuan Y, Zhang S, Chen W et al.. Associations between TNFSF13B polymorphisms and primary Sjögren's syndrome susceptibility in primary Sjögren's syndrome patients: A meta-analysis. Immunity, inflammation and disease 2023. link 11 Gong Y, Liu H, Li G, Zhang T, Li Y, Guan W et al.. Childhood-onset primary Sjögren's syndrome in a tertiary center in China: clinical features and outcome. Pediatric rheumatology online journal 2023. link 12 Sun G, Fosbøl EL, Yafasova A, Faurschou M, Lindhardsen J, Torp-Pedersen C et al.. Long-term risk of heart failure and other adverse cardiovascular outcomes in primary Sjögren's syndrome. Journal of internal medicine 2023. link 13 Geng B, Zhang K, Huang X, Chen Y. A meta-analysis of the effect of Sjögren's syndrome on adverse pregnancy outcomes. Clinics (Sao Paulo, Brazil) 2022. link 14 Zhu T, Pan Z, Zhang N. Elevated CXCL13 in primary Sjögren's syndrome and its correlation with disease activity: a systematic review and meta-analysis. Clinical rheumatology 2022. link 15 Robin F, Albert JD, Lescoat A, Martel A, Perdriger A, DeBandt M et al.. Diagnostic Performances of Ultrasound Evaluation of Major Salivary Glands According to the 2019 Outcome Measures in Rheumatology Ultrasound Scoring System. Arthritis care & research 2022. link 16 Yu K, Ying GS, Vivino FB, Gonzales JA, Massaro-Giordano M, Bunya VY. Preliminary Screening Questionnaire for Sjögren's Syndrome in the Rheumatology Setting. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases 2022. link 17 Hao R, Liu Z, Chou Y, Wang Y, Ren X, Jiang X et al.. Impression Cytology and . Frontiers in immunology 2022. link 18 Trevisani VFM, Pinheiro AC, de Magalhães Souza Fialho SC, Fernandes MLMS, Pugliesi A, Pasoto SG et al.. Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic). Advances in rheumatology (London, England) 2022. link 19 Xu X, Shi Y, Yang X, Zhang Y, Qin L, Cai L et al.. Risk factors for hypertension in primary Sjögren's syndrome patients: a nomogram was constructed. Journal of human hypertension 2022. link 20 Lee AS, Scofield RH, Hammitt KM, Gupta N, Thomas DE, Moua T et al.. Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren's. Chest 2021. link 21 Wang ZZ, Zheng QS, Liu HX, Li LJ. Development and Application of the Placebo Response Model in Clinical Trials for Primary Sjögren's Syndrome. Frontiers in immunology 2021. link 22 Price E, Allen A, Rauz S, Tappuni A, Sutcliffe N, Bombardieri M et al.. The management of Sjögren's syndrome: British Society for Rheumatology guideline scope. Rheumatology (Oxford, England) 2021. link 23 García-González M, González-Soto MJ, Gómez Rodríguez-Bethencourt MÁ, Ferraz-Amaro I. The validity of salivary gland scintigraphy in Sjögren's syndrome diagnosis: comparison of visual and excretion fraction analyses. Clinical rheumatology 2021. link 24 Dubost JJ, Couderc M, Pereira B, Mariette X, Seror R, Gottenberg JE et al.. Concomitant fibromyalgia in primary Sjögren's syndrome in the French ASSESS cohort: comparison of the ACR 1990 and ACR 2016 criteria, FiRST questionnaire and physician's opinion. Clinical and experimental rheumatology 2021. link 25 Kaisho J, Egawa G, Kaku Y, Kabashima K. Multiple Annular Eruptions in a Patient with Sjögren's Syndrome: A Quiz. Acta dermato-venereologica 2020. link 26 van Nimwegen JF, Mossel E, Delli K, van Ginkel MS, Stel AJ, Kroese FGM et al.. Incorporation of Salivary Gland Ultrasonography Into the American College of Rheumatology/European League Against Rheumatism Criteria for Primary Sjögren's Syndrome. Arthritis care & research 2020. link 27 Hernández-Molina G, Ávila-Casado C, Hernández-Hernández C, Recillas-Gispert C, Sánchez-Guerrero J. Performance of the 2016 ACR/EULAR SS classification criteria in patients with secondary Sjögren's syndrome. Clinical and experimental rheumatology 2020. link 28 Yoon HJ, Choi W, Yang JM, Ji YS, Lee SS, Yoon KC. Characteristics of dry eye in patients with pre-existing Sjögren's syndrome according to the revised 2016 ACR-EULAR classification criteria. Medicine 2019. link 29 Trevisani VFM, Pasoto SG, Fernandes MLMS, Lopes MLL, de Magalhães Souza Fialho SC, Pinheiro AC et al.. Recommendations from the Brazilian society of rheumatology for the diagnosis of Sjögren's syndrome (Part I): glandular manifestations (systematic review). Advances in rheumatology (London, England) 2019. link 30 Ozisler C, Kaplanoglu H. Evaluation of subclinical atherosclerosis by ultrasound radiofrequency data technology in patients with primary Sjögren's syndrome. Clinical rheumatology 2019. link 31 van Nimwegen JF, van Ginkel MS, Arends S, Haacke EA, van der Vegt B, Sillevis Smitt-Kamminga N et al.. Validation of the ACR-EULAR criteria for primary Sjögren's syndrome in a Dutch prospective diagnostic cohort. Rheumatology (Oxford, England) 2018. link 32 Billings M, Amin Hadavand M, Alevizos I. Comparative analysis of the 2016 ACR-EULAR and the 2002 AECG classification criteria for Sjögren's syndrome: Findings from the NIH cohort. Oral diseases 2018. link 33 Thorne I, Sutcliffe N. Sjögren's syndrome. British journal of hospital medicine (London, England : 2005) 2017. link 34 Torrente-Segarra V, Corominas H, Sánchez-Piedra C, Fernández-Castro M, Andreu JL, Martínez-Taboada VM et al.. Fibromyalgia prevalence and associated factors in primary Sjögren's syndrome patients in a large cohort from the Spanish Society of Rheumatology registry (SJOGRENSER). Clinical and experimental rheumatology 2017. link 35 Le Goff M, Cornec D, Jousse-Joulin S, Guellec D, Costa S, Marhadour T et al.. Comparison of 2002 AECG and 2016 ACR/EULAR classification criteria and added value of salivary gland ultrasonography in a patient cohort with suspected primary Sjögren's syndrome. Arthritis research & therapy 2017. link 36 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. Annals of the rheumatic diseases 2017. link 37 Chen J, Zhao X, Liu H, Zhou S, Yang Y, Li S et al.. A Point-Scoring System for the Clinical Diagnosis of Sjögren's Syndrome Based on Quantified SPECT Imaging of Salivary Gland. PloS one 2016. link 38 Corominas H, Ortiz-Santamaría V, Castellví I, Moreno M, Morlà R, Clavaguera T et al.. Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies. Rheumatology international 2016. link 39 Agarwal A, Sharma SK, Sinha S, Ragesh R, Memon S, Gupta SD et al.. Primary Sjögren's Syndrome Presenting as Flaccid Quadriparesis. The Journal of the Association of Physicians of India 2015. link 40 Cornec D, Saraux A, Cochener B, Pers JO, Jousse-Joulin S, Renaudineau Y et al.. Level of agreement between 2002 American-European Consensus Group and 2012 American College of Rheumatology classification criteria for Sjögren's syndrome and reasons for discrepancies. Arthritis research & therapy 2014. link 41 Fazaa A, Bourcier T, Chatelus E, Sordet C, Theulin A, Sibilia J et al.. Classification criteria and treatment modalities in primary Sjögren's syndrome. Expert review of clinical immunology 2014. link 42 Sankar V, Noll JL, Brennan MT. Diagnosis of Sjögren's syndrome: American-European and the American College of Rheumatology classification criteria. Oral and maxillofacial surgery clinics of North America 2014. link 43 Plešivčnik Novljan M, Rotar Z, Ambrožič A, Vidmar G, Tomšič M. Comparison of the performance of the different classification criteria for primary Sjögren's syndrome: a prospective cohort study. Clinical rheumatology 2014. link 44 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, England) 2014. link 45 Jamilloux Y, Magy L, Hurtevent JF, Gondran G, de Seze J, Launay D et al.. Immunological profiles determine neurological involvement in Sjögren's syndrome. European journal of internal medicine 2014. link 46 Tsuboi H, Hagiwara S, Asashima H, Umehara H, Kawakami A, Nakamura H et al.. Validation of different sets of criteria for the diagnosis of Sjögren's syndrome in Japanese patients. Modern rheumatology 2013. link 47 Oğütcen-Toller M, Gedik R, Gedik S, Göze F. Sjögren's syndrome: a case report and review of the literature. The West Indian medical journal 2012. link 48 Tavoni AG, Baldini C, Bencivelli W, Cavazzini L, Covelli M, De Vita S et al.. Minor salivary gland biopsy and Sjögren's syndrome: comparative analysis of biopsies among different Italian rheumatologic centers. Clinical and experimental rheumatology 2012. link 49 Erhamamci S, Karalezli A, Yilmaz S, Aktas A. The clinical value and histopathological correlation of lacrimal scintigraphy in patients with primary Sjögren's syndrome. Nuclear medicine communications 2012. link 50 Martel C, Gondran G, Launay D, Lalloué F, Palat S, Lambert M et al.. Active immunological profile is associated with systemic Sjögren's syndrome. Journal of clinical immunology 2011. link 51 Güne S, Yilmaz S, Karalezli A, Aktaş A. Quantitative and visual evaluation of salivary and thyroid glands in patients with primary Sjögren's syndrome using salivary gland scintigraphy: relationship with clinicopathological features of salivary, lacrimal and thyroid glands. Nuclear medicine communications 2010. link 52 Kovács L, Szodoray P, Kiss E. Secondary tumours in Sjögren's syndrome. Autoimmunity reviews 2010. link 53 Kondo T, Inoue H, Usui T, Mimori T, Tomimoto H, Vernino S et al.. Autoimmune autonomic ganglionopathy with Sjögren's syndrome: significance of ganglionic acetylcholine receptor antibody and therapeutic approach. Autonomic neuroscience : basic & clinical 2009. link 54 Ng KP, Isenberg DA. Sjögren's syndrome: diagnosis and therapeutic challenges in the elderly. Drugs & aging 2008. link 55 Yaguchi H, Houzen H, Kikuchi K, Hata D, Ura S, Takeda T et al.. An autopsy case of Sjögren's syndrome with acute encephalomyelopathy. Internal medicine (Tokyo, Japan) 2008. link 56 Giusti L, Baldini C, Bazzichi L, Bombardieri S, Lucacchini A. Proteomic diagnosis of Sjögren's syndrome. Expert review of proteomics 2007. link 57 Nishiyama S, Miyawaki S, Yoshinaga Y. A study to standardize quantitative evaluation of parotid gland scintigraphy in patients with Sjögren's syndrome. The Journal of rheumatology 2006. link 58 Houghton K, Malleson P, Cabral D, Petty R, Tucker L. Primary Sjögren's syndrome in children and adolescents: are proposed diagnostic criteria applicable?. The Journal of rheumatology 2005. link 59 Hocevar A, Tomsic M, Praprotnik S, Hojnik M, Kveder T, Rozman B. Parasympathetic nervous system dysfunction in primary Sjögren's syndrome. Annals of the rheumatic diseases 2003. link 60 Friedman JA, Miller EB, Huszar M. A simple technique for minor salivary gland biopsy appropriate for use by rheumatologists in an outpatient setting. Clinical rheumatology 2002. link 61 Vivino FB, Gala I, Hermann GA. Change in final diagnosis on second evaluation of labial minor salivary gland biopsies. The Journal of rheumatology 2002. link 62 Taouli B, Brauner MW, Mourey I, Lemouchi D, Grenier PA. Thin-section chest CT findings of primary Sjögren's syndrome: correlation with pulmonary function. European radiology 2002. link 63 Panchovska M, Sheitanov Y, Martinova F. Locus DR in primary Sjögren's syndrome. Bratislavske lekarske listy 2001. link 64 Amft N, Curnow SJ, Scheel-Toellner D, Devadas A, Oates J, Crocker J et al.. Ectopic expression of the B cell-attracting chemokine BCA-1 (CXCL13) on endothelial cells and within lymphoid follicles contributes to the establishment of germinal center-like structures in Sjögren's syndrome. Arthritis and rheumatism 2001. link44:11<2633::aid-art443>3.0.co;2-9) 65 Barendregt PJ, van den Bent MJ, van Raaij-van den Aarssen VJ, van den Meiracker AH, Vecht CJ, van der Heijde GL et al.. Involvement of the peripheral nervous system in primary Sjögren's syndrome. Annals of the rheumatic diseases 2001. link 66 Ramos-Casals M, Font J, García-Carrasco M, Calvo J, Places L, Padilla O et al.. High circulating levels of soluble scavenger receptors (sCD5 and sCD6) in patients with primary Sjögren's syndrome. Rheumatology (Oxford, England) 2001. link 67 Jonsson R, Haga HJ, Gordon TP. Current concepts on diagnosis, autoantibodies and therapy in Sjögren's syndrome. Scandinavian journal of rheumatology 2000. link 68 Terpos E, Angelopoulou MK, Variami E, Meletis JC, Vaiopoulos G. Sjögren's syndrome associated with multiple myeloma. Annals of hematology 2000. link 69 Sibilia J. Ro(SS-A) and anti-Ro(SS-A): an update. Revue du rhumatisme (English ed.) 1998. link 70 Font J, Ramos-Casals M, Cervera R, Bosch X, Mirapeix E, García-Carrasco M et al.. Antineutrophil cytoplasmic antibodies in primary Sjögren's syndrome: prevalence and clinical significance. British journal of rheumatology 1998. link 71 Field EA, Longman LP, Bucknall R, Kaye SB, Higham SM, Edgar WM. The establishment of a xerostomia clinic: a prospective study. The British journal of oral & maxillofacial surgery 1997. link90683-5) 72 McArthur CP, Daniels PJ, Tishk M, Mayberry WE. Dental professional's role in diagnosing Sjögren's syndrome. General dentistry 1997. link 73 Lee LA, Pickrell MB, Reichlin M. Development of complete heart block in an adult patient with Sjögren's syndrome and anti-Ro/SS-A autoantibodies. Arthritis and rheumatism 1996. link 74 Laurie GW, Ciclitira PJ, Ellis HJ, Pogany G. Immunological and partial sequence identity of mouse BM180 with wheat alpha-gliadin. Biochemical and biophysical research communications 1995. link 75 Wu AJ, Fox PC. Sjögren's syndrome. Seminars in dermatology 1994. link 76 Foster HE, Gilroy JJ, Kelly CA, Howe J, Griffiths ID. The treatment of sicca features in Sjögren's syndrome: a clinical review. British journal of rheumatology 1994. link 77 Isenberg DA. Secondary Sjögren's syndrome--the sausage factory revisited. Clinical and experimental rheumatology 1994. link 78 Milburn JM, Kay D, Ridpath C. Pulmonary nodular amyloidosis in a patient with Sjögren's syndrome diagnosed by transthoracic biopsy. The Journal of the Louisiana State Medical Society : official organ of the Louisiana State Medical Society 1994. link 79 Vaikoussis E, Georgiou P, Nomicarios D. Tear mucus ferning in patients with Sjögren's syndrome. Documenta ophthalmologica. Advances in ophthalmology 1994. link 80 Bragoni M, Di Piero V, Priori R, Valesini G, Lenzi GL. Sjögren's syndrome presenting as ischemic stroke. Stroke 1994. link 81 Moutsopoulos HM, Sarmas JH, Talal N. Is central nervous system involvement a systemic manifestation of primary Sjögren's syndrome?. Rheumatic diseases clinics of North America 1993. link 82 Saito I, Nishimura S, Tsubota K, Miyasaka N. Comparison of diagnosis and treatment of Sjögren's syndrome in Japan and United States. Rheumatic diseases clinics of North America 1992. link 83 Markusse HM, Oudkerk M, Vroom TM, Breedveld FC. Primary Sjögren's syndrome: clinical spectrum and mode of presentation based on an analysis of 50 patients selected from a department of rheumatology. The Netherlands journal of medicine 1992. link 84 St Clair EW. Anti-La antibodies. Rheumatic diseases clinics of North America 1992. link 85 Kraus A, Caballero-Uribe C, Jakez J, Villa AR, Alarcón-Segovia D. Raynaud's phenomenon in primary Sjögren's syndrome. Association with other extraglandular manifestations. The Journal of rheumatology 1992. link 86 Youinou P, Pennec YL, Casburn-Budd R, Dueymes M, Letoux G, Lamour A. Galactose terminating oligosaccharides of IgG in patients with primary Sjögren's syndrome. Journal of autoimmunity 1992. link90151-f) 87 de Villiers WJ, Klemp P. Primary Sjögren's syndrome associated with inappropriate antidiuretic hormone secretion. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1991. link 88 Drosos AA, Pennec YL, Elisaf M, Lamour A, Acritidis NC, Jouquan JR et al.. Sjögren's syndrome in patients with the CREST variant of progressive systemic scleroderma. The Journal of rheumatology 1991. link 89 Inase N, Usui Y, Tachi H, Takayama S, Nakayama M, Korenaga M et al.. Sjögren's syndrome with bronchial gland involvement and multiple bullae. Respiration; international review of thoracic diseases 1990. link 90 Vitali C, Bombardieri S. Diagnostic criteria for Sjögren's syndrome: the state of the art. Clinical and experimental rheumatology 1990. link 91 Moutsopoulos HM, Zerva LV. Anti-Ro (SSA)/La (SSB) antibodies and Sjögren's syndrome. Clinical rheumatology 1990. link 92 Youinou P, Pennec YL, Katsikis P, Jouquan J, Fauquert P, Le Goff P. Raynaud's phenomenon in primary Sjögren's syndrome. British journal of rheumatology 1990. link 93 Katsikis PD, Youinou PY, Galanopoulou V, Papadopoulos NM, Tzioufas AG, Moutsopoulos HM. Monoclonal process in primary Sjögren's syndrome and cross-reactive idiotype associated with rheumatoid factor. Clinical and experimental immunology 1990. link 94 Kelly CA, Griffiths ID. Major upper airways obstruction associated with Sjögren's syndrome: a case report and literature review. British journal of rheumatology 1989. link 95 Andonopoulos AP, Lagos G, Drosos AA, Moutsopoulos HM. Neurologic involvement in primary Sjögren's syndrome: a preliminary report. Journal of autoimmunity 1989. link90179-0) 96 Hedgpeth MT, Boulware DW. Pulmonary hypertension in primary Sjögren's syndrome. Annals of the rheumatic diseases 1988. link 97 Binder A, Snaith ML, Isenberg D. Sjögren's syndrome: a study of its neurological complications. British journal of rheumatology 1988. link 98 Janssens L, Van Baarle A, Daelemans R, Verbraeken H. Paraneoplastic Sjögren's syndrome. Clinical rheumatology 1987. link 99 Suárez JC. Lacrimal proteins in Sjögren's syndrome. Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde 1987. link 100 Robson SC, Klemp P, Meyers OL. Central nervous system manifestations of Sjögren's syndrome. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1986. link