{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T13:20:17Z","timestamp":1790601617124,"version":"4.1.0"},"reference-count":34,"publisher":"BMJ","issue":"Suppl 1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2018,9,13]]},"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:p>Sj\u00f6gren\u2019s syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin\u2019s lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.<\/jats:p>","DOI":"10.1136\/rmdopen-2018-000789","type":"journal-article","created":{"date-parts":[[2018,10,19]],"date-time":"2018-10-19T08:53:00Z","timestamp":1539939180000},"page":"e000789","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":43,"title":["Sj\u00f6gren\u2019s syndrome: state of the art on clinical practice guidelines"],"prefix":"10.1136","volume":"4","author":[{"given":"Vasco C","family":"Rom\u00e3o","sequence":"first","affiliation":[{"name":"Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosaria","family":"Talarico","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7451-0271","authenticated-orcid":false,"given":"Carlo Alberto","family":"Scir\u00e8","sequence":"additional","affiliation":[{"name":"Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Vieira","sequence":"additional","affiliation":[{"name":"N\u00facleo S\u00edndrome de Sj\u00f6gren, Liga Portuguesa Contra as Doen\u00e7as Reum\u00e1ticas, Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1193-0097","authenticated-orcid":false,"given":"Tobias","family":"Alexander","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Charit\u00e9 University Hospital Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Baldini","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"},{"name":"Rheumatology Unit, University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques-Eric","family":"Gottenberg","sequence":"additional","affiliation":[{"name":"Service de rhumatologie, H\u00f4pitaux Universitaires de Strasbourg, Centre National de R\u00e9f\u00e9rence des Maladies Syst\u00e9miques et Auto-immunes Rares Grand-Est Sud-Ouest (RESO), INSERM-UMRS 1109, F-67000, Strasbourg, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi","family":"Gruner","sequence":"additional","affiliation":[{"name":"Unidade de Doen\u00e7as Auto-imunes, Medicina 7.2, Hospital Curry Cabral, Centro Hospitalar de Lisboa Central, Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, Centre de R\u00e9f\u00e9rence des Maladies Syst\u00e9miques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Mouthon","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Cochin, Centre de R\u00e9f\u00e9rence Maladies syst\u00e9miques Autoimmunes Rares d\u2019Ile de France, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris Descartes, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Orlandi","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Division of Rheumatology and Scleroderma Unit, AOU Careggi, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Pamfil","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Pineton de Chambrun","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne 2, H\u00f4pital La Piti\u00e9-Salp\u00eatri\u00e8re, Institut E3M, Sorbonne Universit\u00e9, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Taglietti","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, ASST Spedali Civili di Brescia, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasa","family":"Toplak","sequence":"additional","affiliation":[{"name":"Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"van Daele","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Immunology, Erasmus MC, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob M","family":"van Laar","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Bombardieri","sequence":"additional","affiliation":[{"name":"University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Schneider","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Universit\u00e4tsklinikum D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Department of Internal Medicine, Ghent University Hospital, Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Cutolo","sequence":"additional","affiliation":[{"name":"Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Mosca","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"},{"name":"Rheumatology Unit, University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Mariette","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Sud: AP-HP, H\u00f4pitaux Universitaires Paris-Sud; INSERM UMR 1184, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2018,10,18]]},"reference":[{"key":"2025090313493852000_4.Suppl_1.e000789.1","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1056\/NEJMcp1702514","article-title":"Primary Sj\u00f6gren's Syndrome","volume":"378","author":"Mariette","year":"2018","journal-title":"N Engl J Med"},{"key":"2025090313493852000_4.Suppl_1.e000789.2","doi-asserted-by":"crossref","first-page":"16047","DOI":"10.1038\/nrdp.2016.47","article-title":"Sj\u00f6gren syndrome","volume":"2","author":"Brito-Zer\u00f3n","year":"2016","journal-title":"Nat Rev Dis Primers"},{"key":"2025090313493852000_4.Suppl_1.e000789.3","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1038\/nrrheum.2016.100","article-title":"Treatment of primary Sj\u00f6gren syndrome","volume":"12","author":"Saraux","year":"2016","journal-title":"Nat Rev Rheumatol"},{"key":"2025090313493852000_4.Suppl_1.e000789.4","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.rdc.2016.03.001","article-title":"Use of biologics in Sj\u00f6gren's syndrome","volume":"42","author":"Nocturne","year":"2016","journal-title":"Rheum Dis Clin North Am"},{"key":"2025090313493852000_4.Suppl_1.e000789.5","doi-asserted-by":"crossref","DOI":"10.1186\/s13023-017-0676-3","article-title":"European Reference networks for rare diseases: what is the conceptual framework?","volume":"12","author":"H\u00e9on-Klin","year":"2017","journal-title":"Orphanet J Rare Dis"},{"key":"2025090313493852000_4.Suppl_1.e000789.6","unstructured":"European Commission. European reference networks internet. Available from: https:\/\/ec.europa.eu\/health\/ern\/overview_en [accessed 23 Jun 2018]."},{"key":"2025090313493852000_4.Suppl_1.e000789.7","unstructured":"ERN ReCONNET. European reference network on rare and complex connective tissue and musculoskeletal diseases [Internet]. Available from: http:\/\/reconnet.ern-net.eu\/ [accessed 23 Jun 2018]."},{"key":"2025090313493852000_4.Suppl_1.e000789.8","unstructured":"Institute of Medicine (U.S.). Clinical practice guidelines we can trust. Washington, DC: National Academies Press, 2011."},{"key":"2025090313493852000_4.Suppl_1.e000789.9","doi-asserted-by":"publisher","DOI":"10.1503\/cmaj.090449"},{"key":"2025090313493852000_4.Suppl_1.e000789.10","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pmed.1000097","article-title":"Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement","volume":"6","author":"Moher","year":"2009","journal-title":"PLoS Med"},{"key":"2025090313493852000_4.Suppl_1.e000789.11","article-title":"Characterization of systemic disease in primary Sj\u00f6gren's syndrome: EULAR-SS task force recommendations for articular, cutaneous, pulmonary and renal involvements","volume":"28","author":"Ramos-Casals","year":"2015","journal-title":"Rheumatology"},{"key":"2025090313493852000_4.Suppl_1.e000789.12","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1002\/acr.22968","article-title":"Treatment guidelines for rheumatologic manifestations of Sj\u00f6gren's syndrome: use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain","volume":"69","author":"Carsons","year":"2017","journal-title":"Arthritis Care Res"},{"key":"2025090313493852000_4.Suppl_1.e000789.13","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1016\/j.rbr.2015.07.004","article-title":"Recommendations for the treatment of Sj\u00f6gren's syndrome","volume":"55","author":"Valim","year":"2015","journal-title":"Rev Bras Reumatol"},{"key":"2025090313493852000_4.Suppl_1.e000789.14","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/kex375","article-title":"The British society for rheumatology guideline for the management of adults with primary Sj\u00f6gren\u2019s syndrome","volume":"56","author":"Price","year":"2017","journal-title":"Rheumatology"},{"key":"2025090313493852000_4.Suppl_1.e000789.15","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1586\/1744666X.2016.1109449","article-title":"Early diagnosis of primary Sj\u00f6gren's syndrome: EULAR-SS task force clinical recommendations","volume":"12","author":"Brito-Zer\u00f3n","year":"2016","journal-title":"Expert Rev Clin Immunol"},{"key":"2025090313493852000_4.Suppl_1.e000789.16","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtos.2014.12.001"},{"key":"2025090313493852000_4.Suppl_1.e000789.17","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.adaj.2015.11.008","article-title":"Clinical practice guidelines for oral management of sj\u00f6gren disease: dental caries prevention","volume":"147","author":"Zero","year":"2016","journal-title":"J Am Dent Assoc"},{"key":"2025090313493852000_4.Suppl_1.e000789.18","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2018-eular.7776","article-title":"SP0159 Eular recommendations for the management of sj\u00d6gren\u2019s syndrome","volume":"77","author":"Retamozo","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2025090313493852000_4.Suppl_1.e000789.19","doi-asserted-by":"publisher","DOI":"10.1136\/ard.61.6.554"},{"key":"2025090313493852000_4.Suppl_1.e000789.20","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21591"},{"key":"2025090313493852000_4.Suppl_1.e000789.21","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/art.39859","article-title":"2016 American College of Rheumatology\/European League Against Rheumatism Classification Criteria for Primary Sj\u00f6gren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts","volume":"69","author":"Shiboski","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"2025090313493852000_4.Suppl_1.e000789.22","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2009.110619"},{"key":"2025090313493852000_4.Suppl_1.e000789.23","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.143743"},{"key":"2025090313493852000_4.Suppl_1.e000789.24","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2014-000022","article-title":"EULAR Sj\u00f6gren's syndrome disease activity index (ESSDAI): a user guide","volume":"1","author":"Seror","year":"2015","journal-title":"RMD Open"},{"key":"2025090313493852000_4.Suppl_1.e000789.25","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2014-206008"},{"key":"2025090313493852000_4.Suppl_1.e000789.26","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2015-208504"},{"key":"2025090313493852000_4.Suppl_1.e000789.27","doi-asserted-by":"publisher","DOI":"10.1002\/art.22658"},{"key":"2025090313493852000_4.Suppl_1.e000789.28","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken164"},{"key":"2025090313493852000_4.Suppl_1.e000789.29","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-210448"},{"key":"2025090313493852000_4.Suppl_1.e000789.30","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2016-000364","article-title":"Salivary gland ultrasound abnormalities in primary Sj\u00f6gren's syndrome: consensual US-SG core items definition and reliability","volume":"3","author":"Jousse-Joulin","year":"2017","journal-title":"RMD Open"},{"key":"2025090313493852000_4.Suppl_1.e000789.31","unstructured":"ERN-ReCONNET Disease Info Toolbox (SS). Available from: http:\/\/reconnet.ern-net.eu\/disease_SS\/#1528108857968-34c0017f-022e"},{"key":"2025090313493852000_4.Suppl_1.e000789.32","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.clim.2017.08.009","article-title":"The unmet need in rheumatology: reports from the targeted therapies meeting 2017","volume":"186","author":"Winthrop","year":"2018","journal-title":"Clin Immunol"},{"key":"2025090313493852000_4.Suppl_1.e000789.33","unstructured":"Fisher B , Zeher M , W-F N . The novel anti-CD40 monoclonal antibody CFZ533 shows beneficial effects in patients with primary sj\u00f6gren\u2019s syndrome: a phase iia double-blind, placebo-controlled randomized trial. Arthritis & Rheumatology;69:2538."},{"key":"2025090313493852000_4.Suppl_1.e000789.34","unstructured":"HarmonicSS. HARMONIzation and integrative analysis of regional, national and international Cohorts on primary Sj\u00f6gren\u2019s Syndrome (pSS) towards improved stratification, treatment and health policy making. Available from: http:\/\/harmonicss.eu\/ [accessed 27 Jun 2018]."}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2018-000789","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T20:49:45Z","timestamp":1756932585000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2018-000789"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10]]},"references-count":34,"journal-issue":{"issue":"Suppl 1","published-online":{"date-parts":[[2018,10,18]]},"published-print":{"date-parts":[[2018,10]]}},"alternative-id":["10.1136\/rmdopen-2018-000789"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2018-000789","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10]]}}}