The Immunopathogenesis of Primary SjÖgren'S Syndrome

Abstract

The findings of this study demonstrate that anti-La(SS-B) is a specific marker of primary Sjögren's syndrome and proceeds clinical expression of sicca. In addition, it defines a group of patients with rheumatoid factor, hypergammaglobulinemia and the phenotype HLA-B8. Reactivity of anti-La (SS-B) with a nucleoprotein which associates with two small ribonucleic acids (RNAs) encoded by Epstein-Barr virus (EBV) suggests that EBV may be implicated in the pathogenesis of primary Sjögren's syndrome. Defective cell-mediated immunity to EBV in patients with anti-La could be a factor contributing to the loss of immunological tolerance to the La nucleoprotein during EBV infection when many copies of the EBV-encoded RNAs would be available for association with the La nucleoprotein which would then be recognised as a “foreign” antigen. Primary Sjögren's syndrome following EBV infection has been reported in two cases and awaits further confirmation.