title: Primary Sjögren's syndrome: pathophysiological, clinical and therapeutic advances.
abstract: Primary Sjögren's syndrome was long a vexing dilemma, as the underlying pathophysiological mechanisms remained obscure, disease activity was challenging to evaluate, and no specific treatments were known to be effective. However, recent years have witnessed major advances in these three areas. Convincing evidence has been obtained that innate immunity, most notably mediated by the interferons, plays a role in the initial B-cell activation. A disease activity score was developed during a consensus conference sponsored by the EULAR. Even more importantly, B-cell depletion constitutes a highly promising therapeutic approach. Thus, rituximab was effective in two controlled clinical trials.
authors.affiliation: Service de rhumatologie, centre national de référence pour les maladies auto-immunes systémiques rares, hôpitaux universitaires de Strasbourg, 1, avenue Molière, 67000 Strasbourg, France. jacques-eric.gottenberg@chru-strasbourg.fr
authors.forename: J-E
authors.initials: JE
authors.lastname: Gottenberg
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001402:B-Lymphocytes / Q000276:immunology; D018849:Controlled Clinical Trials as Topic; D006304:Health Status; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008212:Lymphocyte Depletion; D000069283:Rituximab; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000276:immunology* / Q000503:physiopathology* / Q000628:therapy
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab