title: Progress and regression within primary Sjögren's syndrome.
abstract: The purpose of this review is to give a modern view and an update of important areas in primary Sjögren's syndrome (SS), which may be the most common of the autoimmune systemic rheumatic diseases. Interest in aspects of primary SS including clinical manifestations, pathogenesis, aetiology, treatment, prognosis, etc has increased during the past three decades, the volume of scientific papers and the number of theses being the indicators. However, only a fraction of the money that is used for research into rheumatoid arthritis (RA) is used for SS, and the statement that SS is under-diagnosed, under-treated and under-researched will still be valid for several years to come. The topics that are focused on in this review are: (a) clinical areas with subsections on signs and symptoms, terminology, predictors for development of non-Hodgkin malignant lymphoma (NHML) and prognosis, (b) treatment, (c) the Danger model (aetiopathogenesis) and (d) pathology, including immunoglobulin G4 (IgG4)-positive cells.
authors.affiliation: Outpatients Department, Sjögren's Syndrome Research Centre, Malmö, Sweden. Manthorpe@inet.uni2.dk
authors.forename: R
authors.initials: R
authors.lastname: Manthorpe
authors.forename: A
authors.initials: A
authors.lastname: Bredberg
authors.forename: G
authors.initials: G
authors.lastname: Henriksson
authors.forename: A
authors.initials: A
authors.lastname: Larsson
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001964:Bromhexine / Q000627:therapeutic use; D005100:Expectorants / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008954:Models, Biological; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D005100:Expectorants; D007155:Immunologic Factors; D000069283:Rituximab; D001964:Bromhexine