Immunological tests in primary Sjögren syndrome

Abstract

Patients with primary Sjögren syndrome (SS) produce a wide variety of autoantibodies directed at specific nuclear or cytoplasmic antigens. In some cases, the target antigen is present within specific tissues (Table 28.1). B-lymphocyte hyperactivation, the most typical etiopathogenic abnormality of primary SS, accounts for these autoantibodies. Autoantibodies have traditionally been central to classification criteria for SS. The 1993 European Criteria [1] included the presence of one or more of the following four antibodies: antinuclear antibodies (ANA), rheumatoid factor, anti-SSA/Ro, and anti-SSB/La. However, in the 2002 Criteria [2], only anti-SSA/Ro and anti-SSB/La antibodies were included. Together with a positive salivary gland biopsy, the presence of these autoantibodies became mandatory criteria for the classification of primary SS.