ABSTRACT
Sjögren’s syndrome (SS) is a chronic disease characterized by severe dryness of the eyes (keratoconjunctivitis sicca), dryness of the mouth (xerostomia) and evidence of systemic autoimmune disease. SS may exist as a primary condition (primary SS, 1° SS) or as a secondary condition (2° SS) in association with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or progressive systemic sclerosis (PSS). In some 1° SS patients, there may be involvement of the extraglandular organs, including skin, kidney, liver, lung and nervous system. Further-more, these patients may develop a lymphoproliferative syndrome that includes increased risk of lymphoma. Thus, SS provides an opportunity to look at the molecular biology of the interactions of the immune, neurological and exoocrine systems.
