Early diagnosis of primary Sjögren's syndrome: EULAR-SS task force clinical recommendations
Brito-Zerón, Pilar; Theander, Elke; Baldini, Chiara; Seror, Raphaèle; Retamozo, Soledad; Quartuccio, Luca; Bootsma, Hendrika; Bowman, Simon J; Dörner, Thomas; Gottenberg, Jacques-Eric; Mariette, Xavier; Bombardieri, Stefano; de Vita, Salvatore; Mandl, Thomas; Ng, Wan-Fai; Kruize, Aike A; Tzioufas, Athanasios; Vitali, Claudio; Buyon, Jill; Izmirly, Peter; Fox, Robert; Ramos-Casals, Manuel; Eular Sjögren Syndrome Task Force
(2016) Expert Review of Clinical Immunology, volume 12, issue 2, pp. 137 - 56
(Article)
Abstract
Sjögren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The
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European League Against Rheumatism (EULAR) has promoted and supported an international collaborative study group (EULAR-SS Task Force) aimed at developing consensual recommendations to provide a homogeneous approach to the patient with primary SjS presenting with systemic involvement. This review summarizes the key factors that should be taken into account in the diagnostic approach in a patient with suspected SjS according to the main clinical patterns of presentation, and is especially focused on organ-specific systemic disease presentations, including a consensus set of recommendations in order to reach an early diagnosis. Close collaboration with the different specialties involved through a comprehensive multidisciplinary approach is essential in SjS patients presenting with systemic involvements.
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Keywords: Sjögren syndrome, anti-Ro/SS-A antibodies, systemic activity, ESSDAI, classification criteria, focal lymphocytic sialadenitis, early diagnosis, Journal Article, Research Support, Non-U.S. Gov't, Review
ISSN: 1744-666X
Publisher: Expert Reviews Ltd.
(Peer reviewed)