Sjögren's syndrome: state of the art on clinical practice guidelines. (18th October 2018)

• Record Type: Journal Article
• Authors: Romão, Vasco C; Talarico, Rosaria; Scirè, Carlo Alberto; Vieira, Ana; Alexander, Tobias; Baldini, Chiara; Gottenberg, Jacques-Eric; Gruner, Heidi; Hachulla, Eric; Mouthon, Luc; Orlandi, Martina; Pamfil, Cristina; Pineton de Chambrun, Marc; Taglietti, Marco; Toplak, Natasa; van Daele, Paul; van Laar, Jacob M; Bombardieri, Stefano; Schneider, Matthias; Smith, Vanessa; Cutolo, Maurizio; Mosca, Marta; Mariette, Xavier
• Is Part Of: RMD open. Volume 4(2018)Supplement 1
• Publication Date: 2018-10-18
• Subjects: Sjögren's syndrome -- European Reference Networks -- ERN ReCONNET -- clinical practice guidelines -- unmet needs; Musculoskeletal system -- Diseases -- Periodicals; Rheumatism -- Periodicals; 616.7005
• DOI: 10.1136/rmdopen-2018-000789
• Languages: English
• ISSNs: 2056-5933

Abstract

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.