title: Sjögren syndrome.
abstract: Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be observed in children, men and the elderly. The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma. The mechanism underlying the development of SjS is the destruction of the epithelium of the exocrine glands, as a consequence of abnormal B cell and T cell responses to the autoantigens Ro/SSA and La/SSB, among others. Diagnostic criteria for SjS include the detection of autoantibodies in patient serum and histological analysis of biopsied salivary gland tissue. Therapeutic approaches for SjS include both topical and systemic treatments to manage the sicca and systemic symptoms of disease. SjS is a serious disease with excess mortality, mainly related to the systemic involvement of disease and the development of lymphomas in some patients. Knowledge of SjS has progressed substantially, but this disease is still characterized by sicca symptoms, the systemic involvement of disease, lymphocytic infiltration to exocrine glands, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies and the increased risk of lymphoma in patients with SjS.
authors.affiliation: Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Spain.|Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona, Spain.|Department of Autoimmune Diseases, ICMiD, Hospital Clínic, C/Villarroel, 170, 08036 Barcelona, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Brito-Zerón
authors.affiliation: Rheumatology Unit, University of Pisa, Pisa, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Baldini
authors.affiliation: Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Hendrika
authors.initials: H
authors.lastname: Bootsma
authors.affiliation: Rheumatology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
authors.forename: Simon J
authors.initials: SJ
authors.lastname: Bowman
authors.affiliation: Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Bergen, Norway.|Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
authors.forename: Roland
authors.initials: R
authors.lastname: Jonsson
authors.affiliation: Université Paris Sud, INSERM, Paris, France.|Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.affiliation: Oklahoma Sjögren's syndrome Center of Research Translation, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA.
authors.forename: Kathy
authors.initials: K
authors.lastname: Sivils
authors.affiliation: Department of Rheumatology, Malmö University Hospital, Lund University, Lund, Sweden.
authors.forename: Elke
authors.initials: E
authors.lastname: Theander
authors.affiliation: Department of Pathophysiology, School of Medicine, National University of Athens, Athens, Greece.
authors.forename: Athanasios
authors.initials: A
authors.lastname: Tzioufas
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona, Spain.|Department of Autoimmune Diseases, ICMiD, Hospital Clínic, C/Villarroel, 170, 08036 Barcelona, Spain.|Department of Medicine, University of Barcelona, Barcelona, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
mesh_terms: D000906:Antibodies / Q000032:analysis / Q000097:blood; D003937:Diagnosis, Differential; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis / Q000097:blood; D008223:Lymphoma / Q000209:etiology; D012469:Salivary Glands / Q000473:pathology; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy*
chemical_list: D000906:Antibodies; D007136:Immunoglobulins