title: Treating the Underlying Pathophysiology of Primary Sjögren Syndrome: Recent Advances and Future Prospects.
abstract: Sjögren Syndrome (SS) is a systemic autoimmune disease with a wide clinical spectrum that extends from sicca symptoms of the mucosal surfaces to extra-glandular systemic manifestations. Understanding of the pathophysiology of primary SS has advanced over recent years, and this, in turn, has presented new targeted treatment options. We provide a brief, up-to-date description of the pathophysiology of SS and the main etiopathogenic pathways implicated in the disease process and review clinical evidence in support of new treatment options targeting these pathways, highlighting successes and failures, and concluding with a summary of gaps in knowledge and where future research should be focused. Direct and indirect B-cell targeted therapies are currently the most promising biological agents in primary SS, especially for systemic involvement, but other pathways (T-cell co-stimulation, cytokine-based therapies, intracellular pathways and gene therapies) are under development. The next 10 years may witness a disruptive therapeutic scenario in primary SS.
authors.affiliation: Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Spain.|Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, 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: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Barcelona, Spain.|Centro Médico de Córdoba, Hospital Privado, Córdoba, Argentina.
authors.forename: Soledad
authors.initials: S
authors.lastname: Retamozo
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Barcelona, Spain.
authors.forename: Hoda
authors.initials: H
authors.lastname: Gheitasi
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, CELLEX-IDIBAPS, Barcelona, Spain. mramos@clinic.ub.es.|Department of Autoimmune Diseases, ICMiD, Hospital Clínic, C/Villarroel, 170, 08036, Barcelona, Spain. mramos@clinic.ub.es.|Department of Medicine, University of Barcelona, Barcelona, Spain. mramos@clinic.ub.es.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
mesh_terms: D000818:Animals; D001402:B-Lymphocytes / Q000187:drug effects; D016503:Drug Delivery Systems / Q000379:methods; D006801:Humans; D012859:Sjogren's Syndrome / Q000188:drug therapy* / Q000473:pathology*