title: Early diagnosis of primary Sjögren's syndrome: EULAR-SS task force clinical recommendations.
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 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.
authors.affiliation: a Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Department of Autoimmune Diseases, ICMiD , Hospital Clínic , Barcelona , Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Brito-Zerón
authors.affiliation: b Department of Rheumatology , Skane University Hospital Malmö, Lund University , Sweden.
authors.forename: Elke
authors.initials: E
authors.lastname: Theander
authors.affiliation: c Rheumatology Unit , University of Pisa , Pisa , Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Baldini
authors.affiliation: d Department of Rheumatology , Assistance Publique-Hopitaux de Paris, Hôpital Bicêtre, Le Kremlin Bicêtre , Paris , France.|e Department of Rheumatology , Université Paris-Sud, Le Kremlin Bicêtre , Paris , France.
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.affiliation: a Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Department of Autoimmune Diseases, ICMiD , Hospital Clínic , Barcelona , Spain.|f Hospital Privado Centro Médico , Córdoba , Argentina.
authors.forename: Soledad
authors.initials: S
authors.lastname: Retamozo
authors.affiliation: g Clinic of Rheumatology, Department of Medical and Biological Sciences , University Hospital Santa Maria della Misericordia , Udine , Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Quartuccio
authors.affiliation: h Department of Rheumatology and Clinical Immunology , University Medical Center Groningen, University of Groningen , Groningen , the Netherlands.
authors.forename: Hendrika
authors.initials: H
authors.lastname: Bootsma
authors.affiliation: i Rheumatology Department , University Hospitals Birmingham NHS Foundation Trust , Birmingham , UK.
authors.forename: Simon J
authors.initials: SJ
authors.lastname: Bowman
authors.affiliation: j Rheumatology Department , Charité, University Hospital , Berlin , Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Dörner
authors.affiliation: k Department of Rheumatology , Strasbourg University Hospital, Université de Strasbourg , Strasbourg , France.
authors.forename: Jacques-Eric
authors.initials: JE
authors.lastname: Gottenberg
authors.affiliation: d Department of Rheumatology , Assistance Publique-Hopitaux de Paris, Hôpital Bicêtre, Le Kremlin Bicêtre , Paris , France.|e Department of Rheumatology , Université Paris-Sud, Le Kremlin Bicêtre , Paris , France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.affiliation: c Rheumatology Unit , University of Pisa , Pisa , Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Bombardieri
authors.affiliation: g Clinic of Rheumatology, Department of Medical and Biological Sciences , University Hospital Santa Maria della Misericordia , Udine , Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: de Vita
authors.affiliation: b Department of Rheumatology , Skane University Hospital Malmö, Lund University , Sweden.
authors.forename: Thomas
authors.initials: T
authors.lastname: Mandl
authors.affiliation: l Musculoskeletal Research Group, Institute of Cellular Medicine , Newcastle University , Newcastle upon Tyne , UK.
authors.forename: Wan-Fai
authors.initials: WF
authors.lastname: Ng
authors.affiliation: m Department of Rheumatology and Clinical Immunology , University Medical Center Utrecht , Utrecht , the Netherlands.
authors.forename: Aike A
authors.initials: AA
authors.lastname: Kruize
authors.affiliation: n Department of Pathophysiology , School of Medicine, University of Athens , Greece.
authors.forename: Athanasios
authors.initials: A
authors.lastname: Tzioufas
authors.affiliation: o Istituto San Giuseppe , Como and Casa di Cura Lecco , Lecco , Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Vitali
authors.affiliation: p Division of Rheumatology, Department of Medicine , New York University School of Medicine , New York , NY , USA.
authors.forename: Jill
authors.initials: J
authors.lastname: Buyon
authors.affiliation: p Division of Rheumatology, Department of Medicine , New York University School of Medicine , New York , NY , USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Izmirly
authors.affiliation: q Department of Rheumatology , Scripps Memorial Hospital-XiMED , La Jolla , CA , USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Fox
authors.affiliation: a Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Department of Autoimmune Diseases, ICMiD , Hospital Clínic , Barcelona , Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
mesh_terms: D003937:Diagnosis, Differential; D003955:Diagnostic Tests, Routine / Q000379:methods; D042241:Early Diagnosis; D005060:Europe; D006801:Humans; D017410:Practice Guidelines as Topic; D012216:Rheumatic Diseases / Q000175:diagnosis* / Q000628:therapy; D012307:Risk Factors; D012680:Sensitivity and Specificity; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000628:therapy
keywords: ESSDAI; Sjögren syndrome; anti-Ro/SS-A antibodies; classification criteria; early diagnosis; focal lymphocytic sialadenitis; systemic activity