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RMD Open. 2018 Oct 18;4:e000789. doi: 10.1136/rmdopen-2018-000789. eCollection 2018.

Sjögren's syndrome: state of the art on clinical practice guidelines.

RMD open

Vasco C Romão, Rosaria Talarico, Carlo Alberto Scirè, Ana Vieira, Tobias Alexander, Chiara Baldini, Jacques-Eric Gottenberg, Heidi Gruner, Eric Hachulla, Luc Mouthon, Martina Orlandi, Cristina Pamfil, Marc Pineton de Chambrun, Marco Taglietti, Natasa Toplak, Paul van Daele, Jacob M van Laar, Stefano Bombardieri, Matthias Schneider, Vanessa Smith, Maurizio Cutolo, Marta Mosca, Xavier Mariette

Affiliations

  1. Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
  2. Rheumatology Unit, AOU Pisana, Pisa, Italy.
  3. Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
  4. Núcleo Síndrome de Sjögren, Liga Portuguesa Contra as Doenças Reumáticas, Lisbon, Portugal.
  5. Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
  6. Rheumatology Unit, University of Pisa, Pisa, Italy.
  7. Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques et Auto-immunes Rares Grand-Est Sud-Ouest (RESO), INSERM-UMRS 1109, F-67000, Strasbourg, France.
  8. Unidade de Doenças Auto-imunes, Medicina 7.2, Hospital Curry Cabral, Centro Hospitalar de Lisboa Central, Lisbon, Portugal.
  9. Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France.
  10. Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
  11. Department of Clinical and Experimental Medicine, Division of Rheumatology and Scleroderma Unit, AOU Careggi, University of Florence, Florence, Italy.
  12. Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
  13. Service de Médecine Interne 2, Hôpital La Pitié-Salpêtrière, Institut E3M, Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Paris, France.
  14. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili di Brescia, Brescia, Italy.
  15. Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
  16. Department of Internal Medicine and Immunology, Erasmus MC, Rotterdam, The Netherlands.
  17. Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
  18. University of Pisa, Pisa, Italy.
  19. Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
  20. Department of Rheumatology and Department of Internal Medicine, Ghent University Hospital, Ghent, Belgium.
  21. Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.
  22. Université Paris-Sud: AP-HP, Hôpitaux Universitaires Paris-Sud; INSERM UMR 1184, Le Kremlin Bicêtre, France.

PMID: 30402274 PMCID: PMC6203093 DOI: 10.1136/rmdopen-2018-000789

Abstract

Sjögren's 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's 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.

Keywords: ERN ReCONNET; European Reference Networks; Sjögren’s syndrome; clinical practice guidelines; unmet needs

Conflict of interest statement

Competing interests: None declared.

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