Progress and regression within primary Sjogren's syndrome

The purpose of this review is to give a modern view and an update of important areas in primary Sjogren's syndrome ( SS), which may be the most common of the autoimmune systemic rheumatic diseases ( 1). Interest in aspects of primary SS including clinical manifestations, pathogenesis, aetiology, treatment, prognosis, etc ( 2) 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 ( 3) 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.